Issue 35Issue No. 35, Jan 2021
Electronic ISSN 2521-3881
   
Issue No. 35 - Jan 2021
 
 
7-13 Apoptotic Genes of Bax, Bad, Bcl2, and P53 in A549 Lung Cancer Cells Comparison of the Effect of Echinophora platyloba DC. Extract and Cordia myxa L Extract on the Expression of Apoptotic Genes of Bax, Bad, Bcl2, and P53 in A549 Lung Cancer Cells

Mahshad Kalantari1, Maliheh Entezari1, Abolfazl Movafagh2, Kiavash Hushmandi3, Hossein Dehghani4

1 Department of Genetics, Faculty of advanced Sciences and Technology, Tehran Medical Sciences, Islamic Azad University, Tehran, Iran.
2 Department of Medical Genetics, Cancer Research Center, School of Medicine ,Shahid Beheshti University of Medical Sciences, Tehran, Iran.
3 Department of Food Hygiene and Quality Control, Division of Epidemiology and Zoonoses, Faculty of Veterinary Medicine, University of Tehran, Tehran, Iran.
4 Department of Medical Laboratory Sciences, Islamic Azad University, Tehran Medical sciences Tehran Iran

 
  Abstract
 

Introduction: Lung cancer is the most deadly and sumptuous cancer across the globe. Cancer occurrence is increasing progressively and there is no ideal cure yet. Therefore, new therapeutic areas are needed. The use of herbal extracts due to its properties such as antioxidant activity, anti-proliferative effect, and few side effects can be promising in the treatment of cancer. This study aimed to compare the effect of Echinophora platyloba DC. and Cordia myxa L extracts on apoptosis induction in A549 cancer cells.

 

Materials and Methods: In this experiment, the A549 cell line was first cultured in DMEM medium containing 10% FBS and then treated with different concentrations of both compounds. MTT assay was performed to determine IC50 and to compare the viability of cells treated with different concentrations of Echinophora platyloba DC. and Cordia myxa L seed on days 1, 3 and 5. QRT-PCR test was used to investigate the effects of Echinophora platyloba DC. and Cordia myxa L with IC50 on apoptosis induction.

 

Results: MTT results showed that both plant extracts resulted in cell death and decreased viability of lung cancer cells. But the percentage of viability decreased by Echinophora platyloba DC. was more. Also, Echinophora platyloba DC. significantly increased the expression of Bax, P53 and Bad apoptotic genes and decreased the expression of Bcl2 gene, which induces apoptotic death and the cytotoxic effect of Echinophora platyloba DC. over Cordia myxa L.

 

Conclusion: In comparing the effects of these two extracts Echinophora platyloba DC. was more effective than Cordia myxa L and had greater cytotoxicity on A549 cancerous cells in a lesser concentration and could be an appropriate drug candidate for the treatment of lung cancer.

 

Keywords: Lung cancer, Echinophora platyloba DC, Cordia myxa L, Apoptosis

 

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2 14-20

The effect of body mass index on clinico-pathological features and survival outcome in metastatic bladder carcinoma

   

Amrallah A. Mohammed1, Ayman elshentenawy2, Fifi Mostafa Elsayed3

1 Medical Oncology Department, Faculty of Medicine, Zagazig University, Egypt
1 King Salman Armed Forces Hospital-Tabuk City, KSA.
2 Kasr EL Einy center of clinical oncology and nuclear medicine (NEMROCK), Cairo university, Egypt.
3 Clinical Oncology & Nuclear medicine Department faculty of medicine Suez Canal University, Egypt

     
    Abstract
   

Objective: The The study proposed to assess the relation between the body mass index (BMI) and clinicopathological features of metastatic urinary bladder cancer (uBCa) and the influence on survival outcome.

 

Methods: A retrospective study included 201 metastatic uBCa patients. They classified into three groups according to BMI, group I; a BMI of 18.5-24.9 kg/m2, group II; a BMI of 25-29.9 kg/m2, and group III; BMI ≥ 30 kg/m2. The Kaplan - Meier curve used for survival analysis.

 

Results: 69 patients (34.3%) belonged to group I, 75 patients (37.3%) belonged to group II, and 57 patients (28.4%) belonged to group III. Smoking history was detected in 44.8% of patients with Performance Status (PS) 0 in 55.2%, and PS 1 in 26.9%. Of note, 44.8% of patients responded to 1st chemotherapy and 50.7% received more than 2 lines. Through the univariate analysis, poor prognostic outcome was associated with male (P= 0.01), smoking (P=0.002), BMI group II and group III (p=0.00), PS 2 compared with PS 0 (P<0.001), metastasis to liver, lung, and lymph node (P<0.001), and no response to first line chemotherapy (P<0.001). While no effect for age (P=0.1), bone metastasis (P=0.6), serum LDH (P=0.1), serum albumin (P=0.4), and ≥2 chemotherapy lines (P=0.5) on survival outcome. After the follows-up period, the OS was 12.7 months for all patients. Regarding the BMI groups, the median OS was 23.5 months, 12.9 months, and 10.2 months for group I, group II, and group III respectively (p=<0.001).

 

Conclusion: High BMI associated with aggressive clinico-pathological features and poor survival outcome in metastatic uBCa.

Keywords: Bladder cancer, metastasis, obesity, cancer survival.

 

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3 21-26 Estimation of Serum Midkine in Oral Squamous Cell Carcinoma and Oral Premalignancy
   

Dr. Anubhuti Shrivastava1, Dr. Abhinav Shrivastava11, Dr. Shreenivas Kallianpur2, Dr. Gaurav Sharma3, Dr. Manisha S Tijare4

1Abhinav Dental Care, Jabalpur, India
2 Dept of Oral Pathology & Microbiology, Century Int. Institute of Dental Sciences & Research Centre,Kasaragod, Kerela, India
3 Dharamshila, Delhi, India
4 Govt. Medical college,Gondia, Maharashtra, India.

     
    Abstract
   

Background: Oral squamous cell carcinoma (OSCC) is an aggressive epithelial malignancy. Diagnosis at an early stage is a key for successful cancer therapy. Development of sensitive, specific, and non-invasive tumor markers, especially, in serum, is needed. Midkine (MK) is a plasma-secreted multifunctional peptide which is a heparin-binding growth factor.

 

Materials and Methods: Blood samples were collected from 20 patients with OSCC, 20 patients with oral premalignancy and 10 healthy controls. Only histologically proven oral cancer and precancerous patients were taken as test subjects. Healthy individuals without predisposing habits were selected. The Human Midkine ELISA kit (Biovendor,Czech Republic) was stored at 2-80C.

 

Statistical Analysis: One way ANOVA was applied using SPSS software.

 

 

Results: Midkine Concentration in Poorly differentiated was significantly higher than Well differentiated OSCC. Midkine Concentration in stage II was significantly higher than stage I. There was a very strong positive and significant correlation between severity of disease and Midkine concentration. Also there was a strong positive and significant correlation between histological grades of oral squamous cell carcinoma and Midkine concentration.

 

Conclusions: MK is a soluble, secreted cytokine and can be quantitated in blood. This is a merit of any biomarker compared to biopsy, as sampling of blood is minimally invasive, convenient, inexpensive and can be performed frequently for detecting, monitoring and managing illness. Increased MK expression in tissues, blood and urine has a strong relationship with higher malignant potential. Serum MK concentration may serve for cancer screening and monitoring the prognosis of the disease.

 

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4 27-35

Impact and Cost-Effectiveness of Oncotype DX for Guiding Adjuvant Chemotherapy Decisions in Early Breast Cancer

   

Fadi El Karak1, Elie Rassy 1, Josiane Bassil 1, Mohamed Awali 1, Colette Hanna 1 , Anthony Saroufim1 , Fadi Nasr1 , Fadi Farhat1 , Joseph Kattan1 , Marwan Ghosn1 , Mike Paulden2

1 Department of Mmedical Oncology, Saint Joseph University, Faculty of Medicine, Beirut, Lebanon
2 School of Public Health, University of Alberta, Canada

     
    Abstract
   

Introduction: Oncotype DX is approved in multiple countries but its cost-effectiveness is a matter of considerable health debate. Lebanon is high-middle income country according to the World Bank classification however it is facing a mounting financial and health care burden from cancer. Therefore, we conducted a costeffectiveness analysis of Oncotype DX based Lebanese on real-life data.

Methods: We updated a Canadian cost-effectiveness model of Oncotype DX by incorporating Lebanese data. The patient population was a real-life cohort of 82 women diagnosed with hormone receptor - positive and HER2 - negative early breast cancer.

Results: Overall, providing Oncotype DX to only intermediate Adjuvant! Online risk patients costs an additional $83 CAD (93,883 LBP) per additional QALY. From this point, extending provision to also cover high Adjuvant! Online risk patients costs an additional $736 CAD (831,578 LBP) per additional QALY. From this point, extending provision further to also cover low Adjuvant! Online risk patients (such that Oncotype DX is provided to all patients) costs an additional $14,562 CAD (16.46m LBP) per additional QALY. Given that most women in our population-based sample were classified as intermediate Adjuvant! Online risk patients, our study focused on this subset in the second analysis. Providing Oncotype DX to intermediate Adjuvant! Online risk patients has a relatively small additional cost compared to not providing Oncotype DX, and results in a relatively large QALY gain. The incremental cost per QALY is $2,022 CAD (2.29m LBP), implying that Oncotype DX is cost-effective for intermediate Adjuvant! Online risk patients if the willingness-to-pay for a QALY is greater than 2.29m LBP.

Conclusion: As one of the few economic evaluations to date conducted using Lebanese data, this evaluation provides information to decision makers regarding the cost-effectiveness of providing Oncotype DX to Lebanese patients.

Keywords: Oncotype DX; Lebanese; cost effectiveness; early stage breast cancer

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5 36-41 Brain Metastasis from Breast Cancer: risk factors and radiotherapy perspective from a tertiary Middle Eastern facility
   

Karzan Marif Murad1, Hazha Abdullah Muhammad2, Hawzheen Aziz Muhammad3 Shkar Othman Arif1, Shwan Ali Mohammed1, Kamaran Ahmad Mohammed1, Jalil Salih Ali1, Bamo Muhammed Muhsin1, Nyan Othman Saeed1, Sazgar Star Majeed1, Layth Mula-Hussain4

1 Radiation Oncology, Zhianawa Cancer Center, Sulaimani, Kurdistan, Iraq
2 Medical Oncology, Hiwa Cancer Hospital - College of Medicine - University of Sulaimani, Sulaimani, Kurdistan, Iraq.
3 Cancer Biology, College of Medicine - University of Sulaimani, Sulaimani, Kurdistan, Iraq
4 Radiation Oncology, The Ottawa Hospital Cancer Centre - Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada

     
    Abstract
   

Introduction: Breast cancer represents the second most frequent cause of brain metastases after lung cancer. Previous studies have identified the subgroups of patients with triple-negative and HER2-positive as having an increased risk for the development of brain metastases. We are not aware in Kurdistan - Iraq of any national studies that are in parallel with these findings.

Patients and Methods: A cross-sectional descriptive study conducted on 57 patients who were known cases of breast cancer with brain metastasis, managed with whole brain radiotherapy at a tertiary radiotherapy institute in two years (January 2015 to December 2016), as a convenient sample. Data were collected from patients' archives and phone calls and then analyzed using SPSS version 23.

Results: Younger age at diagnosis and cancers with HER2-positive receptor phenotype are risk factors for brain metastasis. Median survival post-brain metastasis is significantly affected by receptor phenotypes (2 months in triple negative versus 7 months in hormone receptor positive) and performance status (18 months if performance score of 70% and above versus 1.5 months if it is 60% and less).

Conclusion: Primary breast cancer patients have more risk to develop brain metastases if they are at younger age and HER2-positive and the survival post-brain metastases is dramatically affected by both triple negative receptor phenotype and lower performance score.

Keywords: Breast cancer, Brain metastasis, Radiotherapy,Middle East, Iraq.

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6 42-53 Identification of PKHD1 mutations in Brain, Breast and Rectal tumors by Next Generation DNA Sequencing
   

Duaa Mohammed Almehmadi1,2, Abdulrhman Saleh Dairi2, Amal Ali Hassan3,4, Anas Dannoun5, Hussain Saleh Banni5, Ehab M. Melibary5, Abdel-Rahman Youssef6,7, Mohiuddin M. Taher5,8

1 Department of Internal medicine, King Faisal General Hospital Makkah, Makkah, Saudi Arabia
2 Faculty of Medicine, Umm-Al-Qura University, Makkah, Saudi Arabia,
3 Histopathology Division, Al-Noor Specialty Hospital, Makkah, Saudi Arabia
4 Faculty of Medicine, Department of Pathology, Al Azhar University, Cairo, Egypt.
5 Departments of Medical Genetics, Umm-Al-Qura University, Makkah, Saudi Arabia
6 Department of Basic and Clinical Oral Sciences, Faculty of Dentistry, Umm-Al-Qura University, Makkah, Saudi Arabia.
7 Department of Microbiology, Faculty of Medicine, Suez Canal University, Ismailia, Egypt.
8 Science and Technology Unit, Umm-Al-Qura University, Makkah, Saudi Arabia

     
    Abstract
   

Introduction: It is well established that the PKHD1 mutations are associated with autosomal recessive polycystic kidney disease (ARPKD). Although, PKHD1 mutations are also detected in certain cancer types, to our knowledge in rare tumors such as, atypical teratoid rhabdoid tumor (ATRT), primary neuro-ectodermal tumor (PNET), atypicalchoroid plexus papilloma (a-CPP), amelanotic ano-rectal melanoma (AMM), and breast phyllodes tumors PKHD1 mutations profiling is not reported.

Methods and Methods: In order to determine the PKHD1 gene mutation patterns in the brain, rectal, and breast tumors we have analyzed these tumor DNA by Ion Proton Next generation DNA sequencing.

Results: Next-generation DNA sequencing on Ion Proton identified unique and common missense mutations in the brain, breast and ano-rectal tumors. All mutations were benign, and only one pathogenic mutation in p. (Cys3346Arg) found in AMM tumor. In phyllodes tumor of breast, two unique missense variants were detected (rs113562492) p. (Met2841Val); and (rs137972270) in p. (Arg589Cys) and these variants are not present in other tumors tested. The variant rs137972270 was reported only in two cases sofar in ClinVar database. Missense variants such as rs115045643, rs116809571, rs115338476, and rs76895755 are found only in PNET, and a variant rs62406032 in a-CPP, another one rs35445653 in ATRT cases were unique for these tumors, which are not present in other tumors. Several synonymous and intronic variants of PKHD1 gene were also found in these tumors. A synonymous variant p. (Asp395Asp), rs1896976 and two intronic SNPs viz., rs1326605, and rs1571084 were found in all tumors tested. The SNP rs9395699 in IVS66 was found uniquely in IPC breast tumor only in this study. Allele coverage, allele ratio, p-value, Phred qual score, sequencing coverage, alleles frequencies were also analysed, the p-values and Phred quality score were significantly higher.

Conclusion: These tumors did not have any insertion/ deletion mutations, nonsense, or truncated mutations in it. The screening of PKHD1 gene revealed signature mutations for the solid tumors studied by NGS method. This investigation may help in understanding these tumor pathology at molecular level.

Keywords: Breast cancer, Brain cancer, Autosomal recessive polycystic kidney disease; Polycystic kidney and hepatic disease 1 gene; Next generation DNA sequencing

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7 54-58 Mesenchymal Chondrosarcoma-A Retrospective study
 

Deepthi Beena1, Jayasree Kattoor1, Anitha Mathews1, Sindhu P. Nair1, Venugopal M2, Priyakumari T3, Geetha N4

1 Department of Pathology
2 Radiodiagnosis
3 Pediatric Oncology
4 Medical Oncology, Regional Cancer Centre, Thiruvavanthapuram, Kerala. 695011, India.

     
    Abstract
   

Introduction: Mesenchymal chondrosarcoma is a rare high grade malignant neoplasm that accounts for 3-10% of all chondrosarcomas. Histopathologically, it shows biphasic population composed of small round to ovoid with occasional spindle cells and islands of well differentiated cartilage. The study aimed at retrospectively analysing the clinical, pathological, radiological features of these cases in our institution.

Materials and Methods: This is a retrospective descriptional study. All the cases of mesenchymal chondrosarcomas were retrieved from our archives of pathology over a period of 10 years .The demographic details including the age, clinical presentation including skeletal/extraskeletal along with radiology were noted for all these cases. The treatment details along with the follow up of the patients were archived from the medical records.

Results: A total of 13 cases of mesenchymal chondrosarcoma were retrieved for our study. The mean age of presentation was 33 years with a slight male predilection. Extra skeletal soft tissue origin was noted in 3 of our cases (3/13), one case in forearm, another in pelvis. The third case was intracranial origin which presented as a dural based parieto-occipital mass and rest all had bony origin .The radiological and clinical correlation was done for all these cases.

Conclusion: Mesenchymal chondrosarcoma presents multiple diagnostic challenges, most common include inadequate biopsy samples which may result in errors in diagnosis, namely with small blue round cell tumours .A better understanding of this entity may help the pathologists in conferring an accurate diagnosis to the clinicians.

Keywords: Mesenchymal chondrosarcoma;extra skeletal mesenchymal chondrosarcoma;chondrosarcoma; biphasic pattern

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8 59-65 Evaluation of cardiac toxicity of systemic treatments in Moroccan patients followed for localized breast cancer: Prospective observational study of 549 cases.
   

Mariam Haffadi1, Nezha Tawfiq1, Meriam Dakir1, Mouna Boughafour1, Zineb Bouchbika1, Nadia Benchakroun1, Hassan Jouhadi1, Souha Sahraoui1, Abdelatif Benider1, Ikram Krikez2, Rim Benmallek2, Rachida Habbal2

1 Mohammed VI Cancer Treatment Center, Ibn Rochd University Hospital, Hassan II University, Casablanca, Morocco.
2 Deparment of Cardiology, Ibn Rochd University Hospital, Hassan II University, Casablanca, Morocco.

     
    Abstract
   

Objective: Breast cancer is the most common cancer in women in the world and in Morocco. Anthracyclines and anti-HER2 therapy are major drugs in the therapeutic management of localized breast cancer. The most serious toxicity of these drugs is cardiotoxicity. Our work aims to assess the prevalence of this toxicity in the Moroccan population.

Patients and Methods: We conducted a prospective longitudinal observational study between January 2017 and June 2018. All our patients were followed in The Cardio-Oncology Unit, 1st unit of its kind in Morocco, created thanks to the collaboration between the Mohammed VI Cancer Treatment Center and The Cardiology Departement of Ibn Rochd University Hospital in Casablanca. Eligible patients (n=549) had Stage I-III localized breast cancer, verified histologically, and a pre-treatment adequate cardiac function with a LVEF = 50%, measured with echocardiography, and received systemic cardiotoxic treatment (anthracycines, anti-her2 drugs). All patients received regular monitoring of cardiac function mainly by echocardiography. Cardiotoxicity was defined as a decrease in LVEF of 10 points and / or <50%.

Results: A decrease in LVEF was observed in 8.4% of our patients, with 4% symptomatic heart failure. The baseline average LVEF in the cardiotoxicity group was 63.5% (50-77) versus 60.5% (60-74) in the group without cardiotoxicity. 97.1% of these patients received anthracyclines, 98% received trastuzumab against 97% and 65% in the group without cardiotoxicity respectively. Cardiotoxicity was reversible in 6.4% of patients, permanent discontinuation of cardiotoxic treatment was observed in 2.2%. A statistically significant relationship was found between cardiotoxicity and arterial hypertension (HTA) (p = 0.002), trastuzumab (p = 0.0001) and radiotherapy for left breast cancer (p = 0.023).

Discussion and Conclusion: This is one of the first observational studies in Morocco with a large number of patients, which gives us an idea of the cardiotoxicity of systemic treatments in Moroccan localized breast cancer patients. Our results join those of the literature, but are still worrying and invite us, oncologists and cardiologists, to be more vigilant with this toxicity, which influences the oncological and cardiac prognosis of our patients, especially cancer survivors.

Keywords: Breast cancer, anthracyclines, trastuzumab, cardiotoxicity, left ventricular ejection fraction.

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9 66-76 Transanal total mesorectal excision for patients with rectal cancer : a Systematic review and meta-analysis.
   

Jihane Ziati1,2, Amine Souadka1,2,3, Amine Benkabbou1,2,3, Saber Boutayeb2,3,4, Brahim Ahmadi2,5, Laila Amrani1,2,3, Raouf Mohsine1,2,3, Mohammed Anass Majbar1,2,3

1 Surgical Oncology Department National Institute of Oncology. Rabat, Morocco.
2 Faculté de Médecine. Mohammed V University in Rabat. Morocco.
3 Equipe de recherce en oncologie translationnelle (EROT).Mohammed V University in Rabat. Morocco.
4 Medical Oncology Department. National Institute of Oncology. Rabat, Morocco.
5 Intensive care unit. National Institute of Oncology. Rabat, Morocco.

     
    Abstract
   

Bacground: Transanal total mesorectal excision (TaTME) is a new technique that is designed to overcome the limits encountered during laparoscopic total mesorectal excision (LaTME) for rectal cancer, especially in male, obese patients with a narrow pelvis and mid and low rectal tumours.

Aim: The objective of our meta-analysis is to evaluate short-term oncological and perioperative outcomes of transanal total mesorectal excision (TaTME) compared to laparoscopic total mesorectal excision (LaTME) for rectal cancer.

Methods: A meta-analysis based on Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines was conducted in MEDLINE (PubMed). All original studies published in English that compared TaTME with laTME were included. The quality of the included studies was assessed by the Newcastle- Ottawa Quality Assessment Scale (NOS) and Cochrane Library Handbook 5.1.0. Data analysis was conducted using the Review Manager 5.3 software.

Results: Twelve studies including 835 TaTME patients and 1707 LaTME patients with rectal cancer met the inclusion criteria in this meta-analysis. No statistical significant differences were observed in regard to positive circumferential resection margin (PCRM), positive distal resection margin (PDRM), macroscopic quality of mesorectum (MQM) and harvested lymph nodes (HLN). Concerning the perioperative outcomes, the results of conversion rates, operative time, hospital stay (HS), anastomotic leakage (AL) and postoperative complications were comparable between the two groups.

Conclusion: Our meta-analysis provides that TaTME may be a valid alternative approach for the treatment of rectal cancer in comparison with LaTME.

Keywords: Transanal total mesorectal excision; Laparoscopy; Rectal neoplasms; Total mesorectal excision, Meta-analysis

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10 77-81 Innate and adaptive immune cells in Tumor microenvironment
   

Shrihari T.G.

Department of Oral Medicine and Oral Oncology, Krishnadevaraya College of Dental Sciences, Bengaluru - 562157, India.

     
    Abstract
   

Abstract: Innate and adaptive immune cells recruited to the tumor microenvironment by chemokines following noxious stimuli such as physical or chemical or infectious agents. Innate and adaptive immune cells release inflammatory mediators such as cytokines, chemokines, growth factors, proteolytic enzymes, activate transcription factors (NF-KB, STAT3,HIF-1a) results in tumor initiation, promotion, and progression. This article highlights about the role of innate and adaptive immune cells in tumor microenvironment.

Keywords: Neutrophils, Macrophages, Mast cells, Tregs, Bregs, MDSC

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11 82-85 Primary large cell neuroendocrine carcinoma of the ovary: A rare entity
   

Fatima Safini1, Hassan Jouhadi2, Hicham El Attar3

1 Regional Oncology Center, Agadir, Morocco.
2 Mohammed VI Cancer Treatment Center, Radiation-Oncology Department, Ibn Rochd University Hospital, Casablanca, Morocco.
3 Laboratory of Cytology and Histopathology Moulay Driss 1st, Casablanca, Morocco.

     
    Abstract
   

Background: Large-cell neuroendocrine carcinoma (LCNEC) of the ovary is rare, highly aggressive tumor and diagnosed at advanced stages. Immunohistochemistry is required for the diagnosis. The optimal treatment management is not codified because of its rarity.

Case Presentation: We report the case of a 36-year-old woman with a locally advanced stage LCNEC of the ovary managed by surgery and adjuvant chemotherapy with etoposide and cisplatin. She remained disease free until now four years after the end of chemotherapy.

Conclusion: This report suggests the necessity of immunohistochemical analysis in the diagnosis of LCNEC of the ovary. Due to the rarity of LCNC, the general consensus on treatment is not standardized. We used adjuvant chemotherapy regimen similar to large cell carcinoma of the lung.

Keywords: Large-cell, neuroendocrine, ovary carcinoma.

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12 86-91 Langherhans cell histiocytosis presenting as primary infertility in a young male
   

Chandan Kumar1, Garima Jain1, Hiya Boro2, Deepam Pushpam3, Mukesh Yadav4, Sameer Bakhshi3, Sandeep Mathur5, Pranay Tanwar11

1 Laboratory Oncology Unit, Dr B.R.A. Institute Rotatory Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
2 Department of Endocrinology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
3 Department of Medical Oncology, Dr B.R.A. Institute Rotatory Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India
4 Department of Radiology, Dr B.R.A. Institute Rotatory Cancer Hospital, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India 5 Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India

     
    Abstract
   

Abstract: Langerhans cell histiocytosis (LCH) is a rare clonal neoplastic disorder of Langerhans cells, with an incidence rate of 5 per million individuals. In adults LCH usually affects bone followed by lung, skin, pituitary gland, liver, spleen, and orbits. LCH presenting with endocrinopathy is rare and commonly involves posterior pituitary with central diabetes insipidus (DI). Here, we present a rare case of LCH involving posterior pituitary but presenting as infertility in a 25-year-old married man. Later the thyroid gland was also found to be involved in the form of multiple nodules. Fine needle aspiration cytology (FNAC) from right lobe of thyroid showed sheets of Langerhans cells along with entrapped residual thyroid follicular cells which were further confirmed by immunocytochemistry as well as cell block preparation followed by immunohistochemistry. A final diagnosis of LCH involving pituitary and thyroid was made and patient was then started on treatment according to LCH treatment protocol LCH III-6 consisting of prednisolone and vinblastine (6 weeks with daily 40 mg/m2 oral prednisolone, and 6 mg/m2 i.v. vinblastine every 7 days). Patient is responding well to the therapy and is on follow up.

Keywords: Langarhans cell histiocytosis, pituitary, infertility; thyroid

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13 92-95 Malignant melanoma of urethra in a young female - Case report and Literature review
 

Jahnabi Das1, Nikhila Radhakrishna2, Gunaseelan K3, Bhawana A Badhe4

1 Dept. of Radiation Oncology, Guwahati State Cancer Institute, Assam , India
2 Dept. of Radiation Oncology, Kidwai Memorial Institute of Oncology, Bangalore, India
3 Dept. of Radiation Oncology, Jawaharlal Institute of Post graduation Medical Education & Research (JIPMER), Puducherry
4 Dept. of Pathology, Jawaharlal Institute of Post Graduate Medical Education & Research (JIPMER), Puducherry.

   

    Abstract
   

Abstract: Malignant mucosal melanoma (MM) is a rare and aggressive neoplasm, which behaves differently from its cutaneous counterpart. MM of head and neck account for 55.4%, genital tracts - 18%, ano-rectal - 23.8% and urinary tract - 2.8%. Less than 200 cases of urethral melanomas have been reported in literature, out of which MM of female urethra represent only 0.2% of primary melanomas. Owing to the delayed presentation, early onset of metastasis and aggressive tumor biology, prognosis has remained largely poor despite adequate local control in MM. Surgery is the mainstay of management, with adjuvant radiation contributing to local control but not overall survival benefit. Chemotherapy and immunotherapy options are being explored in both adjuvant and palliative setting. We hereby, report the clinical presentation, management, and course of a rare case of urethral melanoma in a young female patient. After ruling out metastatic disease at presentation, local control was achieved by wide local excision with distal urethrectomy and modified Singapore flap reconstruction along with bilateral modified superficial inguinal block dissection; followed by adjuvant radiotherapy in view of close margins. However, she developed distal recurrence three months post adjuvant therapy, thus reflecting the aggressive biology of this class of rare tumor.

Keywords: Mucosal melanoma, urethra, adjuvant radiotherapy

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