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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.
Timely publication of journal: Publication of manuscripts and bringing out the issue in time is one of the positive aspects of JCDR and is possible with strong support team in terms of peer reviewers, proof reading, language check, computer operators, etc. This is one of the great reasons for authors to submit their work with JCDR. Another best part of JCDR is "Online first Publications" facilities available for the authors. This facility not only provides the prompt publications of the manuscripts but at the same time also early availability of the manuscripts for the readers.
Indexation and online availability: Indexation transforms the journal in some sense from its local ownership to the worldwide professional community and to the public.JCDR is indexed with Embase & EMbiology, Google Scholar, Index Copernicus, Chemical Abstracts Service, Journal seek Database, Indian Science Abstracts, to name few of them. Manuscriptspublished in JCDR are available on major search engines ie; google, yahoo, msn.
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.
It is well said that "happy beginning is half done" and it fits perfectly with JCDR. It has grown considerably and I feel it has already grown up from its infancy to adolescence, achieving the status of standard online e-journal form Indian continent since its inception in Feb 2007. This had been made possible due to the efforts and the hard work put in it. The way the JCDR is improving with every new volume, with good quality original manuscripts, makes it a quality journal for readers. I must thank and congratulate Dr Hemant Jain, Editor-in-Chief JCDR and his team for their sincere efforts, dedication, and determination for making JCDR a fast growing journal.
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
Case Series
Full Version
Diagnostic Challenges of Soft Tissue Sarcomas with Special Emphasis on Immunohistochemical Profile- A Case Series
Correspondence Address :
Manisha Mahata,
Flat-2A, Vasundhara Niwas, P-27, Motijheel Avenue, Dumdum,
Kolkata-700074, West Bengal, India.
E-mail: drmanisha252@gmail.com
Soft tissue tumours arise from the mesenchymal tissues of different origin like adipocytic, smooth muscles, skeletal muscle, fibroblastic/myofibroblastic, fibrohistiocytic, pericytic, vascular, osteo-cartilagineous or even unknown. Its diagnosis depends on the age of the patient and site of presentation but sometimes they may present at unusual sites. Some of the tumours have overlapping histomorphological features which results in a list of differential diagnosis. Therefore, immunohistochemistry and molecular genetics play an important role for definite diagnosis. French Federation of Cancer Centres Sarcoma Group System (FNCLCC) based on necrosis, tumour grading and tumour differentiation is commonly used for grading of the sarcomas to assess the treatment response and prognosis of the patient. Hereby, authors present a series of nine cases of soft issue tumours, comprising five cases of synovial sarcomas and one each of dedifferentiated liposarcoma, rhabdomyosarcoma, epithelioid angiosarcoma and Well Differentiated Liposarcoma (WDLS). Few cases are located at unusual anatomical sites; hence, they should be kept in mind as differential diagnosis. Immunohistochemistry has played an important role for confirmation of diagnosis of synovial sarcomas and angiosarcoma.
Dedifferentiated liposarcoma, Epithelioid angiosarcoma, Rhabdomyosarcoma, Synovial sarcoma, Well differentiated liposarcoma
Soft tissue tumours originate from mesenchymal cells with different differentiation like adipocytic, fibroblastic/myofibroblastic, fibrohistiocytic, pericytic, vascular, osteo-cartilagineous, skeletal and smooth muscles (1). World Health Organisation (WHO) Classification of bones and soft tissue tumours, 2020 further classifies them as benign, intermediate (locally aggressive and rarely metastasising) and malignant (1). Hence, soft tissue tumours are a huge spectrum where clinico-radiological correlation is required along with histology, immunohistochemistry and cytogenetics for a definite diagnosis (2). Here, we present a series of diversified soft tissue tumours encountered in a tertiary healthcare centre of Eastern India.
Case 1
A 55-year-old female presented to the Department of General Surgery with an infra-axillary (9×7) cm intermittently painful swelling for four months. Her medical history was insignificant. Magnetic Resonance Imaging (MRI) thorax revealed a {80 (anterioposterior)x56(mediolateral)x64(superioinferior)}mm well defined, heterointense lesion in the subcutaneous plane of infra-axillary region. Biopsy showed a combination of epithelial and spindle cell elements-epithelial component represented by well-formed glandular structures surrounded by a basement membrane while the spindle cells arranged in fascicles and whorling pattern. Individual cell had scant cytoplasm, ovoid nuclei, vesicular chromatin and inconspicuous nucleoli. The intervening areas of necrosis and approximately 8 mitotis/10 High Power Field (HPF) classified it as a FNCLCC Grade 2 sarcoma (Table/Fig 1)a. The epithelial and sarcomatous component were immunopositive for Epithelial Membrane Antigen (EMA) (Table/Fig 1)b, Pan-Cytokeratin clone AE1/AE3 (Table/Fig 1)c, Transducin-Like Enhancer of split 1 (TLE1) (Table/Fig 1)d and Vimentin (Table/Fig 1)e, CD99 respectively. The histomorphology was in favour of biphasic synovial sarcoma. The patient was treated with chemotherapy and followed-up for four months. There was no recurrence noted.
Case 2
A 20-year-old female presented to the Department of Gynaecology with a (5x4) cm painful swelling in the inguinolabial region. Computed Tomography (CT) scan of pelvis showed a well circumscribed (60×53×31) mm lesion with few calcific foci. Microscopically, uniform spindle cells with scant cytoplasm and dense chromatin arranged in short, tightly packed fascicles were noted. A hemangiopericytomatous pattern and very low mitotic count was noted. Necrosis was absent. It was a FNCLCC Grade 2 sarcoma [Table/Fig-2a,b]. They were CD99, vimentin and EMA positive while CD34, S100 and Smooth Muscle Antibody (SMA) negative. Therefore, diagnosis of monophasic synovial sarcoma was offered. She was treated with ifosfamide and doxorubicin. However, follow-up could not be done as the family was non compliant.
Case 3
A 34-year-old male presented to the Department of General Surgery with an 8 cm gradually increasing swelling over left thigh for five months. Radiologically, it was a soft tissue heterogenous mass with calcific foci. Incisional biopsy showed a spindle cell lesion with few necrotic areas. The tumour cells gave cytoplasmic positivity for EMA and CK while nuclear for TLE1; giving a favourable diagnosis of monophasic synovial sarcoma; later, confirmed by Fluorescent In-Situ Hybridisation (FISH) for t(X:18) (Table/Fig 3). The patient lost to follow-up after wide local excision.
Case 4
A 43-year-old female came to the Department of General Surgery with a 3 cm nodular lump in right leg; associated with intermittent pain. CT scan of leg revealed a well circumscribed heterogenous lesion of soft tissue origin. It was histologically and immunohistochemically similar to the case 1 stated above (Table/Fig 4). So, it was diagnosed as biphasic synovial sarcoma. Wide local excision was done and the patient was doing well till two months.
Case 5
A 29-year-old male presented to the Department of General Surgery complained of a 4 cm rapidly increasing painful swelling in right arm. Tru-cut biopsy cores revealed similar histological and immunohistochemical features as that of case 2 stated above (Table/Fig 5)a,b. The diagnosis of monophasic synovial sarcoma was later confirmed by the fusion study for SYT-SSX-1 gene. Neoadjuvant chemotherapy was given initially; however, he was non compliant and follow-up was lost.
Case 6
A 40-year-old female presented to the Department of General Surgery with abdominal discomfort, weight loss and anorexia for 5 months. Radiological examination showed a (8×7×7) cm retroperitoneal mass with two components differing in signal intensity. Grossly, there were wide necro-haemorrhagic areas and sections from different areas of the mass were submitted. Histology revealed areas of WDLS with abrupt transition to the non lipogenic sarcomatous areas (Table/Fig 6)a,b. The diagnosis of dedifferentiated liposarcoma was given. Surgical excision was done. However, a close follow-up showed a local recurrence after six months.
Case 7
A 51-year-old lady presented to the Department of General Surgery with abdominal discomfort. There was no significant history of radiation exposure. Radiology showed a diffuse (5×3) cm mass in spleen. Biopsy demonstrated large epithelioid cells predominantly in nests and sheets (Table/Fig 7)a. Individual cell had abundant eosinophilic cytoplasm, large vesicular nuclei and prominent macronucleoli. Irregular vascular spaces lined by protuberant epithelioid cells were noted (Table/Fig 7)b. Necrosis and high mitotic count was present. They were CD34 (Table/Fig 7)c and EMA (Table/Fig 7)d immunopositive. Hence, the diagnosis of epithelioid angiosarcoma of spleen was given. No local recurrence or metastasis has been noted after receiving chemotherapy till the date of case reporting after a follow-up for four months.
Case 8
A 14-year-old male came to the Department of General Surgery with a gradually increasing scrotal mass for 8 months. CT scan revealed an enlarged testicular tumour measuring (13×10.5×8.5) cm. The specimen of testis with spermatic cord was received after orchiectomy. Cut section showed a solid-whitish growth measuring (14×11×9) cm with a small area of normal looking testicular area. Histology showed atypical spindle cells admixed with large round ovoid cells resembling rhabdomyoblasts (Table/Fig 8)a,b. Tadpole cells and those with cross striations were also noted. A mitotic rate of >19/10HPF, focal (<50%) necrosis and a differentiation score of 2; FNCLCC grade 3 was assigned. The cells were MyoD1 positive and a histomorphological diagnosis favouring embryonal rhabdomyosarcoma was given. On close follow-up of the case for five months, it was found that he had been doing well after receiving chemotherapy.
Case 9
A 55-year-old male presented to the Department of General Surgery with a painful scrotal swelling for six months. Radiologically, it was a paratesticular mass with homogenous intensity. Grossly, it was a (10×8×6) cm greyish brown to yellow tissue piece. Histopathological sections showed variable sized adipocytes with scattered atypical lipoblasts and stromal cells (Table/Fig 9). Necrosis and mitotic activity was absent. Hence, histopathological diagnosis of WDLS was delivered. Wide local excision was performed and follow-up of the case could not be done.
(Table/Fig 10) summarises the diversified soft tissue sarcomas with respect to their age of the patient, anatomical site and immunohistochemistry.
Soft tissue sarcomas are a group of rare heterogeneous tumours which arises in the connective tissues embryologically derived from the mesenchyme (3). Sarcomas constitute <1% of all neoplasms, which often results in a delay in diagnosis (4). The adverse prognostic factors for STS include increasing age, size, high grade, metastasis at diagnosis, high levels of tumour necrosis, local recurrence at diagnosis (following unplanned excision), positive surgical margin and deep to muscular fascia (5).
Synovial sarcoma: It is a malignant soft tissue tumour of uncertain differentiation. Its relative frequency is age-dependent, being 1.6% in more than 50 years and 15% in the age group of 10-18 years (6). It can virtually be found anywhere in the body, predominantly in the extremities (7). A study by Ferrari A et al., of 271 patients showed that the median age at presentation was 32 years with 232 cases involving the extremities, followed by trunk, head and neck, abdominopelvic region and mediastinum (8). In our study, the age of the patients ranged from 20-55 years; median being 34 years. Three of them were present in the extremities while two cases were located at rare sites like inguinolabial and infra-axillary region. Although the histomorphology is characteristic, the differentials should be considered before giving a definite diagnosis (9),(10). Monophasic subtype should be distinguished from malignant peripheral nerve sheath tumour, fibrosarcoma and solitary fibrous tumour using S100 and CD34 respectively. Biphasic mesothelioma or ectopic hamartomatous thymoma could be considered in the Biphasic subtype (7). Synovial Sarcoma shows positivity with TLE1, CD99, SMA, Vimentin, CK and EMA and fusion study for SYT-SSX-1 gene (1). Hence, immunohistochemistry and molecular studies play an important role in the definite diagnosis of synovial sarcoma.
Dedifferentiated liposarcoma (DL): It is the tumour with a non lipogenic component within an atypical lipomatous tumour. It accounts for 18% of liposarcomas. According to the FNCLCC grading system, dedifferentiated liposarcoma is assigned 3 points for differentiation, resulting in an intermediate grade at the minimum. DLs located in the retroperitoneum have the worst prognosis. Local recurrence has occurred in at least 40% of cases. However, almost all retroperitoneal cases seem to recur locally if patients are followed for 10-20 years (1).
Angiosarcomas: They are highly aggressive malignant vascular tumours constituting less than 1% of all sarcomas (11). The most common locations are the skin, soft tissue, breast, bone, liver and spleen (7). Some soft tissue angiosarcomas arise from major vessels, such as the inferior vena cava or aorta. It is difficult to identify the endothelial nature of the lesion as they have an undifferentiated appearance with a solid growth pattern. The different histologic growth patterns include papillary, spindled and epithelioid morphologies. The common etiologies for angiosarcomas include previously irradiated field, in arteriovenous fistulas or arising within pre-existing benign tumours, such as haemangioma, neurofibroma or leiomyoma. The histomorphology of EA may show diagnostic challenges with other lesions like epithelioid haemangioma, Epithelioid Haemangioendothelioma (EHE), metastatic carcinoma, metastatic melanoma, lymphoma, epithelioid sarcoma and other sarcomas with epithelioid features. More than 50% patients die of this within one year (12).
Rhabdomyosarcomas: They are malignant skeletal muscle tumours and histologically are of three types-embryonal, alveolar and pleomorphic. Embryonal variant constitute approximately 60% (13). The genitourinary tract is the second most common site for rhabdomyosarcoma, following head and neck (14). In the Intergroup Rhabdomyosarcoma Study (IRS) series, 24% cases arose in this region. The tumours most commonly arise in a paratesticular location occurring predominantly in adolescents. Histologically, most tumours present in this location are of the embryonal subtype, spindle cell variant [15,16]. It has an excellent prognosis, with a 5-year overall survival rate of about 90%, when confined disease is treated with combined surgery, radiation and chemotherapy and less than 30%, when metastatic (17).
Atypical lipomatous tumour and WDLS: They are used synonymously. They belong to the Intermediate (locally aggressive) category of adipocytic tumours in the WHO classification of soft tissue and bone, 2020. However, the latter is used for lesions arising in anatomical sites like retroperitoneum, spermatic cord and mediastinum, which have greater potential for disease progression. They account for 30%-40% of all liposarcomas (18). Three main histologic patterns are recognised: lipoma-like, sclerosing and inflammatory (lymphocyte-rich). WDLS is a grade 1 sarcoma in the FNCLCC classification. Recurrences are common because of the difficulty of radical excision. It does not metastasise unless it dedifferentiates (19). Hence, this diagnosis should alert the surgeons to the well-known problem of local recurrence and these tumours should be fully excised (20).
Soft tissue tumours may be of diverse origin. Their diagnosis is an amalgam of clinico-radiological, histomorphological, immunohistochemical and molecular correlation. Proper diagnosis is helpful in predicting the prognosis of the patient. Complete excision is required as few of them have increased risk of recurrence.
DOI: 10.7860/JCDR/2022/53215.16180
Date of Submission: Nov 12, 2021
Date of Peer Review: Dec 09, 2021
Date of Acceptance: Feb 08, 2022
Date of Publishing: Apr 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: Nov 17, 2021
• Manual Googling: Feb 07, 2022
• iThenticate Software: Feb 17, 2022 (7%)
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