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Prof. Somashekhar Nimbalkar
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"Journal of Clinical and Diagnostic Research is at present a well-known Indian originated scientific journal which started with a humble beginning. I have been associated with this journal since many years. I appreciate the Editor, Dr. Hemant Jain, for his constant effort in bringing up this journal to the present status right from the scratch. The journal is multidisciplinary. It encourages in publishing the scientific articles from postgraduates and also the beginners who start their career. At the same time the journal also caters for the high quality articles from specialty and super-specialty researchers. Hence it provides a platform for the scientist and researchers to publish. The other aspect of it is, the readers get the information regarding the most recent developments in science which can be used for teaching, research, treating patients and to some extent take preventive measures against certain diseases. The journal is contributing immensely to the society at national and international level."



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Dr. Arundhathi. S
MBBS, MD (Pathology),
Sanjay Gandhi institute of trauma and orthopedics,
Bengaluru.
On Aug 2018




Dr. Mamta Gupta,
"It gives me great pleasure to be associated with JCDR, since last 2-3 years. Since then I have authored, co-authored and reviewed about 25 articles in JCDR. I thank JCDR for giving me an opportunity to improve my own skills as an author and a reviewer.
It 's a multispecialty journal, publishing high quality articles. It gives a platform to the authors to publish their research work which can be available for everyone across the globe to read. The best thing about JCDR is that the full articles of all medical specialties are available as pdf/html for reading free of cost or without institutional subscription, which is not there for other journals. For those who have problem in writing manuscript or do statistical work, JCDR comes for their rescue.
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Reviewing articles is no less a pain staking process and requires in depth perception, knowledge about the topic for review. It requires time and concentration, yet I enjoy doing it. The JCDR website especially for the reviewers is quite user friendly. My suggestions for improving the journal is, more strict review process, so that only high quality articles are published. I find a a good number of articles in Obst. Gynae, hence, a new journal for this specialty titled JCDR-OG can be started. May be a bimonthly or quarterly publication to begin with. Only selected articles should find a place in it.
An yearly reward for the best article authored can also incentivize the authors. Though the process of finding the best article will be not be very easy. I do not know how reviewing process can be improved. If an article is being reviewed by two reviewers, then opinion of one can be communicated to the other or the final opinion of the editor can be communicated to the reviewer if requested for. This will help one’s reviewing skills.
My best wishes to Dr. Hemant Jain and all the editorial staff of JCDR for their untiring efforts to bring out this journal. I strongly recommend medical fraternity to publish their valuable research work in this esteemed journal, JCDR".



Dr. Mamta Gupta
Consultant
(Ex HOD Obs &Gynae, Hindu Rao Hospital and associated NDMC Medical College, Delhi)
Aug 2018




Dr. Rajendra Kumar Ghritlaharey

"I wish to thank Dr. Hemant Jain, Editor-in-Chief Journal of Clinical and Diagnostic Research (JCDR), for asking me to write up few words.
Writing is the representation of language in a textual medium i e; into the words and sentences on paper. Quality medical manuscript writing in particular, demands not only a high-quality research, but also requires accurate and concise communication of findings and conclusions, with adherence to particular journal guidelines. In medical field whether working in teaching, private, or in corporate institution, everyone wants to excel in his / her own field and get recognised by making manuscripts publication.


Authors are the souls of any journal, and deserve much respect. To publish a journal manuscripts are needed from authors. Authors have a great responsibility for producing facts of their work in terms of number and results truthfully and an individual honesty is expected from authors in this regards. Both ways its true "No authors-No manuscripts-No journals" and "No journals–No manuscripts–No authors". Reviewing a manuscript is also a very responsible and important task of any peer-reviewed journal and to be taken seriously. It needs knowledge on the subject, sincerity, honesty and determination. Although the process of reviewing a manuscript is a time consuming task butit is expected to give one's best remarks within the time frame of the journal.
Salient features of the JCDR: It is a biomedical, multidisciplinary (including all medical and dental specialities), e-journal, with wide scope and extensive author support. At the same time, a free text of manuscript is available in HTML and PDF format. There is fast growing authorship and readership with JCDR as this can be judged by the number of articles published in it i e; in Feb 2007 of its first issue, it contained 5 articles only, and now in its recent volume published in April 2011, it contained 67 manuscripts. This e-journal is fulfilling the commitments and objectives sincerely, (as stated by Editor-in-chief in his preface to first edition) i e; to encourage physicians through the internet, especially from the developing countries who witness a spectrum of disease and acquire a wealth of knowledge to publish their experiences to benefit the medical community in patients care. I also feel that many of us have work of substance, newer ideas, adequate clinical materials but poor in medical writing and hesitation to submit the work and need help. JCDR provides authors help in this regards.
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In the era of fast growing newer technologies, and in computer and internet friendly environment the manuscripts preparation, submission, review, revision, etc and all can be done and checked with a click from all corer of the world, at any time. Of course there is always a scope for improvement in every field and none is perfect. To progress, one needs to identify the areas of one's weakness and to strengthen them.
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Every one of us: authors, reviewers, editors, and publisher are responsible for enhancing the stature of the journal. I wish for a great success for JCDR."



Thanking you
With sincere regards
Dr. Rajendra Kumar Ghritlaharey, M.S., M. Ch., FAIS
Associate Professor,
Department of Paediatric Surgery, Gandhi Medical College & Associated
Kamla Nehru & Hamidia Hospitals Bhopal, Madhya Pradesh 462 001 (India)
E-mail: drrajendrak1@rediffmail.com
On May 11,2011




Dr. Shankar P.R.

"On looking back through my Gmail archives after being requested by the journal to write a short editorial about my experiences of publishing with the Journal of Clinical and Diagnostic Research (JCDR), I came across an e-mail from Dr. Hemant Jain, Editor, in March 2007, which introduced the new electronic journal. The main features of the journal which were outlined in the e-mail were extensive author support, cash rewards, the peer review process, and other salient features of the journal.
Over a span of over four years, we (I and my colleagues) have published around 25 articles in the journal. In this editorial, I plan to briefly discuss my experiences of publishing with JCDR and the strengths of the journal and to finally address the areas for improvement.
My experiences of publishing with JCDR: Overall, my experiences of publishing withJCDR have been positive. The best point about the journal is that it responds to queries from the author. This may seem to be simple and not too much to ask for, but unfortunately, many journals in the subcontinent and from many developing countries do not respond or they respond with a long delay to the queries from the authors 1. The reasons could be many, including lack of optimal secretarial and other support. Another problem with many journals is the slowness of the review process. Editorial processing and peer review can take anywhere between a year to two years with some journals. Also, some journals do not keep the contributors informed about the progress of the review process. Due to the long review process, the articles can lose their relevance and topicality. A major benefit with JCDR is the timeliness and promptness of its response. In Dr Jain's e-mail which was sent to me in 2007, before the introduction of the Pre-publishing system, he had stated that he had received my submission and that he would get back to me within seven days and he did!
Most of the manuscripts are published within 3 to 4 months of their submission if they are found to be suitable after the review process. JCDR is published bimonthly and the accepted articles were usually published in the next issue. Recently, due to the increased volume of the submissions, the review process has become slower and it ?? Section can take from 4 to 6 months for the articles to be reviewed. The journal has an extensive author support system and it has recently introduced a paid expedited review process. The journal also mentions the average time for processing the manuscript under different submission systems - regular submission and expedited review.
Strengths of the journal: The journal has an online first facility in which the accepted manuscripts may be published on the website before being included in a regular issue of the journal. This cuts down the time between their acceptance and the publication. The journal is indexed in many databases, though not in PubMed. The editorial board should now take steps to index the journal in PubMed. The journal has a system of notifying readers through e-mail when a new issue is released. Also, the articles are available in both the HTML and the PDF formats. I especially like the new and colorful page format of the journal. Also, the access statistics of the articles are available. The prepublication and the manuscript tracking system are also helpful for the authors.
Areas for improvement: In certain cases, I felt that the peer review process of the manuscripts was not up to international standards and that it should be strengthened. Also, the number of manuscripts in an issue is high and it may be difficult for readers to go through all of them. The journal can consider tightening of the peer review process and increasing the quality standards for the acceptance of the manuscripts. I faced occasional problems with the online manuscript submission (Pre-publishing) system, which have to be addressed.
Overall, the publishing process with JCDR has been smooth, quick and relatively hassle free and I can recommend other authors to consider the journal as an outlet for their work."



Dr. P. Ravi Shankar
KIST Medical College, P.O. Box 14142, Kathmandu, Nepal.
E-mail: ravi.dr.shankar@gmail.com
On April 2011
Anuradha

Dear team JCDR, I would like to thank you for the very professional and polite service provided by everyone at JCDR. While i have been in the field of writing and editing for sometime, this has been my first attempt in publishing a scientific paper.Thank you for hand-holding me through the process.


Dr. Anuradha
E-mail: anuradha2nittur@gmail.com
On Jan 2020

Important Notice

Case Series
Year : 2022 | Month : May | Volume : 16 | Issue : 5 | Page : ER06 - ER09 Full Version

Diagnostic Challenges of Uncommon Paediatric Head and Neck Masses- A Case Series


Published: May 1, 2022 | DOI: https://doi.org/10.7860/JCDR/2022/56156.16344
Karthik Sigamani, Preethi Srinivasan

1. Associate Professor, Department of Pathology, Karpaga Vinayaga Institute Of Medical Sciences and Research Centre, Chengalpattu, Tamil Nadu, India. 2. Senior Resident, Department of Pathology, Karpaga Vinayaga Institute Of Medical Sciences and Research Centre, Chengalpattu, Tamil Nadu, India.

Correspondence Address :
Dr. Preethi Srinivasan,
Om Nivas, A-21/B, Alagesa Nagar, Chengalpattu-603001, Tamil Nadu, India.
E-mail: om.lovely93@gmail.com

Abstract

Paediatric head and neck masses pose diagnostic challenge to the clinicians owing to the wide spectrum of lesions including congenital (developmental), inflammatory and neoplastic lesions. In this case series, uncommon paediatric head and neck mass lesions that were diagnosed in a tertiary care hospital over a period of three years from January 2017 to December 2019, had been included, emphasizing on the diagnostic challenges encountered. First case was of Cervical Chondrocutaneous Branchial Remnant (CCBR) that was misdiagnosed as branchial cyst clinically. Second case was a lymphoepithelial cyst that masqueraded as an acute suppurative lymphadenitis in Fine Needle Aspiration Cytology (FNAC) due to florid inflammation. Third case was an aggressive poorly differentiated malignant neoplasm of left nasal cavity namely Nuclear protein in Testis (NUT) carcinoma that had to be differentiated from several other small round blue cell tumors. Fourth was a case of lipoblastoma which could be mistaken for myxoid liposarcoma. The last two cases were vascular tumors, one of them was juvenile capillary hemangioma with a benign clinical course while the other was an intermediate grade tumor namely kaposiform hemangioendothelioma. A wholesome clinical, radiological and pathological evaluation will help to solve the diagnostic dilemmas in this group of lesions.

Keywords

Children, Congenital, Tumor, Cyst, Branchial cleft

Paediatric head and neck masses are commonly encountered in clinical practice that pose serious diagnostic challenge to the clinicians owing to the wide spectrum of lesions that can occur in this region in children. Broadly paediatric head and neck lesions are classified into three categories namely congenital (developmental), inflammatory and neoplastic lesions based on etiology (1). Accurate diagnosis is possible only based on combination of proper clinical examination, radiological correlation and histopathological examination. The difficulty lies not only in diagnosis but also in handling parental anxiety and careful management of these lesions without any damage to vital structures in this region of the body of children. Any misdiagnosis can adversely affect the management and prognosis of this wide group of paediatric head and neck lesions. In this case series, uncommon paediatric head and neck mass lesions that were diagnosed in a tertiary care hospital over a period of three years from January 2017 to December 2019 had been included, emphasizing on the diagnostic challenges encountered and their unique features (Table/Fig 1).

Case Report

Case 1

A 2-year-old male child reported to the Department of General Surgery with a chief complaint of painless mass of size 2×2 cm in the right lateral side of neck since birth. There were no other significant complaints and there was no family history of similar swelling in the family members. On clinical examination, the swelling was non tender and soft in consistency. Ultrasonogram (USG) neck impression was branchial cyst following which the lesion was excised and sent for histopathological examination. Grossly, skin covered soft tissue mass measured 3×2.5×1.5 cm with a grey white cut surface. Microscopic examination revealed stratified squamous lining epithelium with a central solid core of mature cartilaginous tissue extending down to the muscles. The mature cartilage was surrounded by lobules of mature adipocytes and skin adnexal structures. Final diagnosis of Cervical Chondrocutaneous Branchial Remnant (CCBR) was made (Table/Fig 1),(2). The follow-up period was uneventful.

Case 2

A 11-year-old female presented in the Department of General Surgery with a swelling of size 3×2 cm in the right lateral side of neck for six months duration associated with pain for past one month. Based on the radiological (ultrasound of neck) impression of abscess and initial Fine Needle Aspiration Cytology (FNAC) report of acute suppurative lymphadenitis, the patient was treated with a course of antibiotics following which pain subsided however there was no reduction in the size of the swelling. Hence, repeat FNAC was done and it showed cellular smears with singly scattered anucleate squames along with mature squamous epithelial cells and sheets of mixed inflammatory infiltrate. Based on these features, a revised diagnosis of branchial cyst was given in FNAC following which excision biopsy was carried out. On gross examination, skin covered soft tissue mass measured 3×1.5×0.7 cm and the cut section revealed a cystic cavity filled with pultaceous material. Microscopic examination showed a cyst lined by stratified squamous epithelium with underlying fibrocollagenous stroma containing diffuse infiltrate of lymphocytes forming follicles with prominent germinal centres. Histopathological features were consistent with lymphoepithelial cyst (branchial cyst) (Table/Fig 1), (Table/Fig 3)a,b. The patient came for follow-up after two months of surgery and there were no complications or recurrence in the follow-up period.

Case 3

A 10-year-old female child presented in the Department of Otorhinolaryngology with a slowly growing painless mass of size 1.5 cm in diameter in the left nasal cavity associated with nasal obstruction for three months duration. Clinically and radiologically (computed tomography scan), the mass was diagnosed as nasal polyp following which excision of the mass was performed. On gross examination, multiple grey white soft tissue fragments altogether measured 0.5cc in aggregate. Microscopic examination of the excisional biopsy showed a neoplasm composed of small uniform round cells arranged in diffuse sheets with scant cytoplasm, round nuclei and coarse chromatin. Focal areas showed characteristic abrupt keratinisation. All these findings were suggestive of a small round blue cell tumor with the differentials of Nuclear protein in Testis (NUT) carcinoma, Ewing sarcoma/ Primitive Neuro Ectodermal Tumor (PNET), olfactory neuroblastoma and sinonasal undifferentiated carcinoma. Immunohistochemistry (IHC) showed positivity for pancytokeratin (CK- AE1/AE3). All other markers namely synaptophysin, chromogranin and CD99 were negative. So, on correlating with histopathological features of characteristic abrupt keratinisation along with cytokeratin positivity in IHC, final diagnosis of NUT carcinoma was made (Table/Fig 1), (Table/Fig 4)a,b. The patient was referred to higher centre for further management.

Case 4

A 5-year-old female child presented in the Department of General Surgery with a painless, soft and mobile swelling of size 2×1 cm in the left lateral side of neck for 1 year duration. Excision of the swelling was done with a clinical and radiological (USG neck) diagnosis of cervical lymphadenopathy. Grossly, the soft tissue mass measured 1.5×1×0.5 cm with yellowish cut surface. Histopathological examination showed a tumor composed of mature and immature adipocytes arranged in lobular pattern in a background of myxoid stroma with small number of capillaries. There was no nuclear atypia or increased mitotic activity. Therefore the diagnosis was consistent with lipoblastoma (Table/Fig 1), (Table/Fig 5). Patient was completely normal in the follow-up period after surgery with no recurrence of the swelling.

Case 5

A 11-year-old female child presented in the Department of General Surgery with a reddish, soft and painless swelling in the right lateral side of neck since birth. The swelling was gradually increasing in size for the past two years and attained the present size of 3x2 cm. Clinically, the lesion was provisionally diagnosed as vascular malformation and the patient was subjected to radiological (ultrasound neck) investigation. The radiological impression was hemangioma following which the swelling was excised and sent for histopathological examination. On gross examination, skin covered soft tissue mass measured 4×3×1.5 cm and the cut surface showed a highly vascular reddish circumscribed mass measuring 3×3×2 cm in the dermis. Microscopic examination showed stratified squamous epithelium with underlying vascular lesion arranged in lobules and composed of numerous capillary sized blood vessels lined by plump endothelial cells with inconspicuous lumens. Histopathological features were consistent with that of Juvenile capillary hemangioma. IHC showed CD31 and CD34 positivity thereby confirming the histopathological diagnosis (Table/Fig 1), (Table/Fig 6)a,b. The follow-up period was uneventful.

Case 6

A 8-year-old male child presented in the Department of General Surgery with a violaceous to reddish, soft and painless swelling of size 4x2 cm in the right lateral side of neck for 1 year duration. The swelling was not present at birth and it was gradually increasing in size. A provisional clinical diagnosis of hemangioma was made on correlating with radiological findings (ultrasound neck) following which excision of the mass was done. On gross examination, globular skin covered soft tissue mass measured 4×2.5×2 cm with a grey brown cut surface. Microscopic examination revealed a tumor composed of fascicles and nodular pattern of spindle shaped cells with slit like spaces containing RBCs along with capillary sized vessels separated by myxoid stroma. Immunohistochemistry showed positivity for CD31 and CD34. Final diagnosis of kaposiform hemangioendothelioma was made (Table/Fig 1), (Table/Fig 7)a,b. The follow-up period was uneventful and there was no recurrence of the swelling after three months of surgery.

Discussion

Paediatric head and neck mass lesions are unique and diagnostically challenging compared to that in adult population. A wide spectrum of lesions ranging from non neoplastic to neoplastic lesions can be encountered with low incidence of malignancy in the head and neck region of children unlike that in adults (1).

In this case series, four cases (66%) were reported in females and only two cases (34%) in males with the mean age group of 7.8 years. All the masses were present in the lateral side of neck with a single case involving the nasal cavity and their mean size was 2.6cm. In a study of 281 paediatric neck masses by Gov-Ari E and Hopewell BL, 55% were females, the mean age group was 7.4 years and the mean size of the mass was 2.6 cm (1). All these findings were in concordance with the present study. Out of the 281 cases in the study of Gov-Ari E and Hopewell BL, from United States of America, a wide variety of histopathological distribution of paediatric head and neck lesions were encountered with the most common being congenital lesions followed by inflammatory and neoplastic lesions respectively. The common congenital lesion in their study was thyroglossal cyst followed by branchial cyst (1). In a study of 35 paediatric neck masses by Osifo OD and Ugiagbe EE, from Nigeria, thyroglossal cyst was the most frequent non neoplastic lesion while hodgkin lymphoma was the commonest neoplastic lesion (2). In another study of neck masses in 207 children from China by Xia X et al., thyroglossal cyst was the most common lesion accounting for 31% of the cases (3). In all the above studies by Gov-Ari E and Hopewell BL, (1), Osifo OD and Ugiagbe EE, (2) and Xia X et al., (3), there were no cases of CCBR, juvenile capillary hemangioma, kaposiform hemangioendothelioma, NUT carcinoma or lipoblastoma reported. This highlights the rarity of such paediatric head and neck mass lesions.

Cervical Chondrocutaneous Branchial Remnants (CCBR) are uncommon paediatric developmental lesions. In a study by Woo HY and Kim HS, which included 1096 patients with branchial cleft anomalies, CCBR accounted for only 0.4% of cases (4). The CCBRs most commonly present in the cervical region along the anterior border of the sternocleidomastoid muscle as cysts, fistulae or sinuses (4). Such lesions should be differentiated from other developmental lesions like thyroglossal cyst, thymic cyst, branchial cleft cyst and hamartoma (5). Histologically, CCBR is characterised by skin with underlying central core of mature cartilage surrounded by fat and skin appendages (4). The CCBRs can be external markers of other serious internal congenital anomalies involving cardiovascular system, auditory system, central nervous system, respiratory system etc. Simple surgical excision is curative for this developmental lesion (4).

Branchial cyst (Lymphoepithelial cyst), though a common developmental branchial cleft abnormality in head and neck region of children, it can be mistaken for inflammatory lesions in FNAC due to florid inflammatory infiltrate (6) similar to the present study. About 90% of branchial cysts are lined by squamous epithelium with the presence of subepithelial lymphoid tissue. Sometimes the epithelium overlying the reactive lymphoid tissue is attenuated or absent causing diagnostic difficulties (7). Complete surgical excision of the cyst is curative in most cases with a recurrence rate of 5% due to incomplete surgical cyst excision (7).

Lipoblastoma is a benign soft tissue neoplasm of infants and children that commonly affects extremities with only 14% cases occurring in head and neck region (8). These tumors exhibit characteristic lobular pattern with spectrum of adipocytic maturation including lipoblasts and mature adipocytes along with spindle to stellate cells in a myxoid matrix resembling myxoid liposarcoma. However younger age group, presence of lobular pattern and fibrous septa will help in excluding myxoid liposarcoma (9). Complete surgical excision is often curative for lipoblastoma. However, recurrence rates of 27% for head and neck lipoblastomas have been reported (9).

Vascular lesions of children include several entities including vascular malformations, various types of hemangiomas, hemangioendotheliomas and very rarely angiosarcomas. Juvenile capillary hemangiomas belong to benign proliferative lesions of vascular origin (10). They are clinically characterised by early proliferative and late involutional stages (11). Kaposiform hemangioendothelioma is an intermediate grade vascular tumor of infancy and childhood (12). It has to be differentiated from juvenile hemangiomas since it doesn’t show remission and exhibits poor response to treatment. Histologically, characteristic nodules with glomeruloid aggregates of vascular channels lined by endothelial cells are seen in kaposiform hemangioendothelioma. All vascular lesions express CD31 and CD34 in immunohistochemical study (13).

The NUT Carcinoma is an aggressive poorly differentiated tumor accounting for 18% of the poorly differentiated carcinomas of the sinonasal region (14). Histologically, the tumor consists of sheets of monotonous population of medium-sized cells having round to oval nuclei along with foci of squamous differentiation in the form of “abrupt” keratinization (14). This uncommon tumor poses diagnostic dilemma by resembling sinonasal undifferentiated carcinoma, Ewing sarcoma/ PNET and olfactory neuroblastoma. IHC will help in arriving at the correct diagnosis with NUT1 being the specific marker. The BRD4-NUT fusion is detected by molecular studies in around 67% of cases. The overall prognosis is poor and it is a highly lethal malignancy in children (14).

Conclusion

There is a great variability in the clinico-histopathological profile of paediatric head and neck mass lesions. In addition to the common lesions, it is imperative to be aware of the uncommon lesions that can be encountered in this region which will aid in proper diagnosis and treatment. A multi-modality approach that includes clinical examination, radiological evaluation and histopathological confirmation is required for the management of this challenging group of mass lesions in children.

References

1.
Gov-Ari E, Hopewell BL. Correlation between pre-operative diagnosis and post-operative pathology reading in paediatric neck masses- A review of 281 cases. Int J Pediatr Otorhinolaryngol. 2015;79:02-07. [crossref] [PubMed]
2.
Osifo OD, Ugiagbe EE. Neck masses in children: Etiopathology in a tertiary center. Niger J Clin Pract. 2011;14:232-6. [crossref] [PubMed]
3.
Xia X, Liu Y, Wang L, Xing Z, Yang L, Xie F. Neck masses in children: A 10 year single centre experience in North West China. British Journal of Oral and Maxillofacial Surgery. 2019;57(8):729-33. [crossref] [PubMed]
4.
Woo HY, Kim HS. Clinicopathological characteristics of cervical chondrocutaneous branchial remnant: A single-institutional experience. Int J Clin Exp Pathol. 2017;10:9866-9877.
5.
Dayal, D, Menon, P. Bilateral cervical chondrocutaneous branchial remnants. Indian Pediatr. 2008;45:221.
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DOI and Others

DOI: 10.7860/JCDR/2022/56156.16344

Date of Submission: Mar 07, 2022
Date of Peer Review: Apr 02, 2022
Date of Acceptance: Apr 19, 2022
Date of Publishing: May 01, 2022

AUTHOR DECLARATION:
• Financial or Other Competing Interests: None
• Was informed consent obtained from the subjects involved in the study? Yes
• For any images presented appropriate consent has been obtained from the subjects. Yes

PLAGIARISM CHECKING METHODS:
• Plagiarism X-checker: Mar 09, 2022
• Manual Googling: Mar 21, 2022
• iThenticate Software: Mar 29, 2022 (3%)

ETYMOLOGY: Author Origin

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