Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

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MBBS, MD (Pathology),
Sanjay Gandhi institute of trauma and orthopedics,
Bengaluru.
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Consultant
(Ex HOD Obs &Gynae, Hindu Rao Hospital and associated NDMC Medical College, Delhi)
Aug 2018




Dr. Rajendra Kumar Ghritlaharey

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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.
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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 report
Year : 2011 | Month : December | Volume : 5 | Issue : 8 | Page : 1659 - 1661 Full Version

The Cytology of the Benign Extra- Gastrointestinal Stromal Tumour in the Pouch of Douglas: A Case Report


Published: December 1, 2011 | DOI: https://doi.org/10.7860/JCDR/2011/.1787
B. Devanand, Vadiraj P.

1. Professor, Medical College (VIMS), Bellary - 583104 Karnataka, India. 2. Consultant No.18, KHB Colony Gandhinagar, Near Women’s college Bellary, Karnataka, India - 583 103.

Correspondence Address :
Vadiraj P., Consultant
No.18, KHB Colony
Gandhinagar, Near Women’s college
Bellary, India - 583 103
pyativadiraj@gmail.com

Abstract

Extra-gastrointestinal stromal tumours are rare, non-epithelial, mesenchymal tumours which arise from the soft tissues of the abdomen- mesentry, the omentum and the retroperitoneum. These tumours are histologically and cytologically similar to the stromal tumours of the gastrointestinal tract, they are composed of purely rounded epithelioid cells or short fusiform cells or a mixture of both and are set in a fine fibrillary myxiod background. The cytological features of the imprint smears in a case of benign extra-gastrointestinal stromal tumour of the mesentry, projecting into the pouch of douglas in a 42-year old female, have been described here. The recognition of these tumours is important because of their aggressive biological behaviour. The metastatic potential and the high rate of recurrence of these tumours necessitate the frequent follow up of the patients after a surgical resection.

Keywords

Epithelioid, extra-gastrointestinal, spindle, stromal tumour

Introduction
Extra-gastrointestinal stromal tumours (EGISTs) are very rare tumours, accounting for less than 10% of the stromal tumours which arise in the gastro-intestinal tract (1). They constitute a group of primary non-epithelial mesenchymal tumours which arise outside the gastrointestinal tract in the soft tissues of the abdomen like the mesentry omentum and the retroperitoneum, with the exclusion of the tumours which arise in the gastro-intestinal tract and the exclusion of those having the classic features of leiomyoma, leiomyosarcoma, schwannoma and other fibromatoses. These tumours are believed to arise from the primitive stromal cells which are capable of differentiating into intestinal smooth muscle or neural cells, because some of these tumours show positivity for the smooth muscle and neural markers.

The cytology of a case of benign extra-gastrointestinal tumour in a 42-year-old female has been presented here.

Case Report

A 42-year-old, premenopausal, multiparous female presented with lower abdominal pain of 3 months duration. The general physical examination was normal. The per-abdominal examination revealed no palpable mass, except for tenderness in the right iliac fossa. The per-vaginal examination showed a tender right fornix, a bulky uterus and fullness in the pouch of douglas. The other systems were normal. Ultrasound examination of the lower abdomen showed a right sided pyosalphinx and a hypoechoic mass in the posterior aspect of the uterus. Abdominal hysterectomy with bilateral salphingo-oopherectomy was done. Per-operatively, a solid tumour which measured 6Ă—4 cms, which arose from the mesentry and was protruding into the pouch of douglas, was made out. This mass was not attached to the gastrointestinal tract or to the genitourinary organs. The mass was enucleated and sent separately for histopathological examination.

The mass was well circumscribed and unencapsulated. The cut section showed a smooth, lobulated and tanned appearance withsolid, cystic and haemorrhagic areas. Imprint smears of the unfixed specimen were taken, fixed in ethyl alcohol and stained with the hematonylin- eosin (H and E) stains.

The cytology revealed many tissue fragments which consisted of loose spindle cells in a myxoid fibrillary background. These spindle cells which had ill-defined cytoplasm were arranged as fascicles or palisades, with spindle or cigar shaped nuclei, with pointed or blunt ends, with finely dispersed chromatin and indistinct nucleoli [Table/ Fig 1 and (Table/Fig 2). Areas showing loosely cohesive sheets of oval or polygonal cells with moderate, well defined cytoplasm and round hyperchromatic nuclei with granular chromatin and nucleoli, which represented an epithelioid morphology were seen (Table/Fig 2). Extra cellular, amorphous eosinophilic material with few spindle cells, which represented skenoid fibres (Table/Fig 2) and many stripped nuclei (Table/Fig 3) were seen. The diagnosis of stromal tumour of extra-gastrointestinal origin was suggested.

The haematoxylin and eosin stained paraffin sections showed interlacing fascicles of benign spindle cells with eosinophilic fibrillary cytoplasm and cigar shaped nuclei. There was no nuclear pleomorphism, mitoses or necrosis. A histopathological diagnosis of extra-gastrointestinal stromal tumour was made and it correlated with the cytology. Immunohistochemistry of the tumour showed positivity for CD (1).

Discussion

Stromal tumours which arise outside the gastro-intestinal tract are very rare (1). These tumours arise from the soft tissues of abdomenmesentry, the omentum and theretroperitoneum. EGISTs have been extensively analyzed by Reith et al (1). EGISTs have been documented in various sites like the omentum, the pancreas and the vulvoviginal and the rectovaginal septa (2) .

Extra-gastro-intestinal stromal tumours (EGIST) are cytologically and histologically similar to gastro-intestinal stromal tumours which are cellular spindle cell or epithelioid tumours which express the CD 34 and the CD 117 (c-kit) antigens (5). EGISTs display various lines of differentiation which reflect the elements of the gut wall, showing differentiation towards smooth muscle and neural elements, dual differentiation and those that lack differentiation towards either cell type (6).EGISTs occur commonly in adults, presenting with abdominal pain or are discovered incidentally during a work up for an unrelated condition (1).

Grossly, these tumours tend to be lobulated, nodular, well circumscribed and unencapsulated with a smooth fleshy, whorledsilk appearance on cut sections. Areas of necrosis and haemorrhage are common in the malignant tumours (6).

Morphologically, EGISTs are sub-classified to be of the spindle or epithelioid types (5). The cytology of these tumours have been described by Mills and Contos (7). The aspirate smears are moderately cellular and are composed of both tight three- dimensional aggregates and noncohesive single cells which are dispersed in a relatively clean background. The nuclei are spindle shaped orepithelioid with a smooth nuclear membrane and they are evenly distributed, with finely granular chromatin and inconspicuous nucleoli. The cytoplasm is cyanophilic, delicate and fibrillar, with long tapered ends. Extra-cellular fibrillary matrix material is usually present. Skenoid fibres, which are not common markers in EGISTs, were found in our case. Additional morphological variations like a prominent myxoid matrix, a signet ring cell, granular cell features and oncocytic cytoplasmic features have been described (6).

The spindle type of EGISTs should be differentiated from solitary fibrous tumours, fibromatosis, inflammatory fibroid tumours, schwannomas, leiomyomas and leiomyosarcomas (6). The epithelioid type of EGISTs should be distinguished from carcinomas, neuroendocrine tumours, melanomas and hepatocellular carcinomas (8).

The cytological features which predict the adverse outcomes are mitotic activity, cellularity and necrosis. Cytology can be used to diagnose EGISTs, but it is not reliable for assessing the malignant potential (9). A histopathological examination is mandatory for all cases of EGISTs.

EGISTs are an aggressive group of stromal tumours with a malignant potential and a high rate of recurrence (1), (4). But our case had a benign cytology. The malignant EGISTs metastasize to the lung, liver and other organs (6).

EGISTs are treated by surgical resection and the administration of tyrosine kinase inhibitors, with frequent follow ups to detect the recurrence (6).

To conclude, EGISTs are very rare aggressive tumours with high metastatic potential and a high recurrence rate. Cytology can be used as a confident diagnostic tool to detect these tumours. Histological examination is mandatory for the assessment of the malignancy to detect parameters like cellularity, mitoses, anaplasia, pleomophism and necrosis.

References

1.
Reith JD, Goldblum JR, Lyles RH and Weiss SW. Extra-gastrointestinal (soft tissue) stromal tumours: An analysis of 48 cases with an emphasis on the histological predicton of the out come. Mod Pathol 2000; 13(5): 577-85.
2.
Sang MC, Mancho S, Trang AW, Goiman B, Almaroof B and Ahmed MY. A malignant omental extragastrointestinal stromal tumour in a young man: a case report and review of literature. Wor J Surg oncol 2008; 6:50 doi: 10.
3.
Padhi. S, Kongara R, Uppin SG, Uppin MS, Prayaga AK, Challa S et al. Extra-gastrointestinal stromal tumour arising in the pancreas: A case report with a review of the literature. J Pancreas (online) 2010;11(3) :244-8.
4.
Lam MM, Corlen CL, Goldblum JR, Heinrich MC, Downs-kelly E, Rubin BP. Extra-gastrointestinal stromal tumours presenting as vulvovaginal / rectovaginal septal masses: a diagnostic pitfall. Int J. Gynecol Pathol 2006; 25(3):288-92
5.
Deshpande A, Munshi MM. Gastro-intestinal stromal tumours-report of 2007; 24: 96-100.
6.
Rosai J. Gastro-intestinal System: Stomach In Juan Rosai editors, Rosai and Ackerman’s Surgical Pathology 9th ed. Vol1. India: Thomson Press, Mosby (Elsevier); 2004; 674-78
7.
Mills AS, Contos MJ, Goel R. The stomach: In Silverberg SG, DeLellis RA, Frable WJ, LiVolsi VA, Wick MR editors. Silverberg’s Principles and Practice of Surgical Pathology and Cytopathology 4th ed. Vol 2 China : Churchill Livingstone (Elsevier) : 2006; 1345-8.
8.
Dong Q, Mckee G, Pitman M, Geisinger K Tambouret R. Epithelioid variant of gastrointestinal stromal tumour: Diagnasis by fine-needle aspiration. Diagn Cytopathol 2003;29:55-60.
9.
Gu M, Ghafari S, Nguyen PT et al. Cytologic diagnosis of gastrointestinal stromal tumours of the stomach by endoscopic, ultrasound-guided, fine-needle aspiration biopsy. Cytomorphological and immuno histochemical study of 12 cases. Diagn cytopathol 2001; 25: 343-50.

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