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Gastrointestinal stromal tumours (GISTs) are rare - and rectovaginal extragastrointestinal stromal tumours (RV-EGISTs) even rarer. We share a case of RV-EGIST, complemented by high-quality radiological and surgical images. A review of current literature pertaining to RV-EGIST is also included. Our case report highlights the diagnostic challenge presented by extragastrointestinal stromal tumours. Differentiated from overlapping pathologies only by targeted application of immunohistopathology and cytogenetics, the inclusion of RV-EGIST in the differential diagnosis of a rectovaginal tumour is essential to making this correct diagnosis. Primary surgery is the treatment of choice for RV-EGIST if complete cytoreduction can be achieved, combined with adjuvant tyrosine kinase inhibitor (TKI) therapy for those with high-risk features to further reduce rates of future recurrence.

Mixed germ cell-sex cord stromal tumours (MGC-SCSTs) of the testis and ovary differ significantly in their histological appearance, clinical behaviour, and molecular genetics. Until recently, the germ cells of testicular MGC-SCST were considered to be invariably histologically bland, whereas those from neoplasms that arise in the ovary have histological features characteristic of premalignancy. However, a recent histological and molecular genetic study demonstrated histological abnormalities and multiple chromosomal losses and gains in a small subset of testicular cases, thus providing the first evidence that testicular MGC-SCSTs can exceptionally show histological and molecular abnormalities. All cases of testicular MGC-SCST reported to date have been clinically benign, whereas ovarian examples are sometimes the precursor of a malignant germ cell neoplasm that can be clinically aggressive. Both genetic and epigenetic influences likely account for dissimilarities in these uncommon gonadal neoplasms.

Molecular profiling was performed in 50 problematic ovarian sex cord-stromal tumours (SCSTs) most of which were seen in consultation. Following analysis, 17 were classified as adult granulosa cell tumour (AGCT), 16 of which showed a FOXL2 sequence variant (mutation); the initial favoured diagnosis in five of the cases was benign thecoma/fibrothecoma. Thirteen tumours ultimately classified as cellular fibroma or thecoma were FOXL2 sequence variant negative which was helpful in excluding AGCT. All six Sertoli-Leydig cell tumours (SLCTs) demonstrated DICER1 'hot spot' sequence variants, and one case each of AGCT and SLCT showed high grade histological transformation associated with a concurrent TP53 sequence variant. All eight unclassified SCSTs were negative for FOXL2 mutations and the six tested cases were DICER1 wild type; however, three tumours demonstrated MET, CTNNB1 or TP53 sequence variants. Four cases were classified as juvenile granulosa cell tumour, and one of these harboured a GNAS sequence variant. The single gynandroblastoma and microcystic stromal tumours in the series demonstrated FOXL2 and CTNNB1 alterations, respectively. In summary, molecular analysis aids in accurate classification of challenging ovarian SCSTs and sometimes leads to revision of the favoured provisional diagnosis. TP53 sequence variants may be associated with dedifferentiation in both SLCTs and AGCTs.

Background and Objectives: The objective was to assess the efficacy and safety in treating gastric stromal tumours by laparoscopy combined with gastroscopy positioning surgery. Methods: The randomised controlled trials (RCTs), which are about the efficacy and safety of laparoscopy combined with gastroscopy positioning surgery in treating gastric stromal tumours were searched from the PubMed (1998–1990–2018.6), Wanfang Data (1990–2018.6), China National Knowledge Infrastructure (1979–2018.6) and International Statistical Institute (1998–2018.6). The data were extracted from these trials, and the meta-analysis was made through from RevMan 5.3 software. Results: Six RCTs involving 451 patients were included in the study (227 patients in the laparoscopy combined with gastroscopy positioning group and 224 patients in laparoscopic surgery group). Compared with laparoscopic surgery group, this meta-analysis showed that laparoscopy combined with gastroscopy positioning group could shorten the post-operation hospital stay (P < 0.05) and reduce the intraoperative blood loss (P < 0.05). However, there was no significant difference in others between the two groups, such as operation time (P > 0.05), post-operative time of recovery of intestinal peristalsis (P > 0.05) and the total hospital stay (P > 0.05). Conclusion: Compared with laparoscopic surgery group, the better total effect occurs in laparoscopy combined with gastroscopy positioning group for the treatment of gastric stromal tumours is better. Laparoscopy combined with gastroscopy positioning group for the gastric stromal tumours is acceptable.

Background Gastro-Intestinal Stromal tumours (GISTs) are rare neoplasms of mesenchymal origin that arise in the GI tract and account for approximately 0.1-3% of all GI tumours. The aim of this study was to determine whether the mode of clinical presentation of GISTs has an effect on outcome. Methods Consecutive 143 patients diagnosed with GISTs were identified from the regional network database (median age 67 (21-88) years, 85 male). Data were collected on presenting symptoms, tumour, treatment undertaken, and the primary outcome measure was survival. Results The commonest GIST anatomical sites were stomach (69.9%), small bowel (13.3%), and colon (4.9%). Commonest symptoms were abdominal pain (30.0%), dyspepsia (11.8%), and anaemia (11.1%). Symptomatic presentations were associated with older age (p = 0.056) and higher mitotic index (>5 per 50HPF, p = 0.045). On univariable analysis the factors associated with cumulative 10-year survival were age (p = 0.076), the presence of symptoms (78.1% vs. 96.4%, p = 0.028), mitotic index (p = 0.011), and modified National Institutes of Health (NIH) prognostic index (p = 0.010), but not deprivation index, anatomical site or GIST diameter. Symptomatic patients aged over 60 years with mitotic index >5 per 50 HPF had the poorest 10-year survival at 63.6% (HR 2.577, 95%CI 1.132-5.867). On multivariable analysis, NIH index (HR 4.283, 95%CI 1.395-13.149, p = 0.011) and age (HR1.061, 95%CI 1.006-1.118, p = 0.029) were independently associated with 10-year survival. Conclusions Age, symptoms, and NIH criteria represent the most important prognostic biomarkers in patients diagnosed with GISTs.

The majority of gastric neoplasms are of epithelial type. Stromal tumours are the next most frequent and are most commonly gastrointestinal stromal tumours, followed by leiomyoma and schwannoma. We present an exceptional case of a patient with a gastrointestinal stromal tumour with suspicion of residual gastric disease, which was diagnosed post-operatively as a schwannoma.

Background and Objectives: The aim of the study was to assess the efficacy and safety in treating gastric stromal tumours by laparoscopy combined with gastroscopy positioning surgery. Methods: I searched the randomized controlled trials (RCTs) about the efficacy and safety of laparoscopy combined with gastroscopy positioning surgery in treating gastric stromal tumours from the PubMed (1998～2018.06), Wanfang Data (1990～2018.06), China National Knowledge Infrastructure (1979～2018.06) and International Statistical Institute (1998～2018.06). I extracted the data from these trials, and I got the meta-analysis from RevMan 5.3 software. Results: Twenty-six RCTs involving 1710 patients were included (870 patients in the laparoscopy combined with gastroscopy positioning group and 840 patients in openresection group). Compared with open resection group, this meta-analysis showed that laparoscopy combined with gastroscopy positioning group could reduce the intraoperative blood (P < 0.05), shorten the post-operative time of recovery of intestinal peristalsis (P < 0.05), shorten the diet recovery time (P < 0.05), reduce the incidence of the incision infection, intestinal obstruction and pneumonia and also shorten the post-operation hospital stay (P < 0.05). However, there was no significant difference in the operation time (P > 0.05). Conclusion: Compared with open resection group, the total effect of laparoscopy combined with gastroscopy positioning group in the treatment of gastric stromal tumours is better. Laparoscopy combined with gastroscopy positioning group for the gastric stromal tumours is acceptable.

Background Ovarian sex cord stromal tumours (OSCSTs) are rare ovarian tumours and include different histopathologic subtypes. This study aimed to analyse the clinical and sonographic characteristics of different histopathologic OSCST subtypes. Methods A total of 63 patients with surgically proven OSCSTs were enrolled in this retrospective study to analyse their clinical and sonographic features. Ultrasound examinations and predictive models were performed before surgery. The clinical and sonographic findings were compared according to the type of OSCST based on the histopathological diagnosis. Results The mean age of 63 patients was 52.17 years (range: 17–78 years). Eighteen patients experienced irregular vaginal bleeding (28.57% 18/63), 7 patients exhibited abnormal body hair (11.11%). 2 patients (3.17%) showed an increased level of CA125, and 25 patients (39.68%, 25/63) showed an increased level of testosterone. Forty-two patients had ovarian thecoma-fibroma groups (OTFGs). Six patients had Sertoli-Leydig cell tumours (S-LCTs), 4 patients had Leydig cell tumours (LCTs), 8 patients had ovarian granulosa cell tumours (OGCTs), 2 patients had ovarian steroid cell tumours, not otherwise specified (OSCTs-NOS), and one patient had sclerosing stromal tumours (SSTs). The mean diameter of the tumour was 47.9 mm (range: 10–258 mm). Forty-seven masses were hypoechoic (74.60%). Twenty-eight masses had posterior echo attenuation, 22 masses exhibited abundant Doppler flow signals (34.92%), and one patient had ascites (1.59%). The diagnostic accuracy of the Simple Rules (SR) and the Assessment of Different NEoplasias in the adneXa (ADNEX) model in distinguishing benign and malignant OSCSTs was 44% (30/63) and 84% (53/63), respectively. The diagnostic accuracy of the SR for OTFGs, S-LCTs & LCTs & OSCTs-NOS, OGCTs, and SSTs was 47.6% (20/42), 16.67% (2/12), 100% (8/8), and 0% (0/1), respectively. The diagnostic accuracy of the ADNEX model for OTFGs, S-LCTs & LCTs & OSCTs-NOS, OGCTs, and SSTs was 93% (31/42), 58.33% (7/12), 75% (6/8), and 100% (1/1), respectively. Conclusions OSCSTs generally appear as a solid mass on ultrasound. Posterior echo attenuation indicates an OTFG. A solid mass with abundant Doppler flow signals indicates an S-LCT, LCT, OSCT-NOS or OGCT. Current predictive models are not very effective, but symptoms, sonographic features and serum hormones are helpful for diagnosis.

Objectives: We developed the first German evidence- and consensus-based clinical guideline on diagnosis, treatment, and follow-up of germ cell tumours (GCT) of the testes in adult patients. We present the guideline content in 2 separate publications. The present second part summarizes therecommendations for the treatment of advanced disease stages and for the management of follow-up and late effects. Materials and Methods: An interdisciplinary panel of 42 experts including 1 patient representative developed the guideline content. Clinical recommendations and statements were based on scientific evidence and expert consensus. For this purpose, evidence tables for several review questions, which were based on systematic literature searches (last search in March 2018), were provided. Thirty-one experts, who were entitled to vote, rated the final clinical recommendations and statements. Results: Here we present the treatment recommendations separately for patients with metastatic seminoma and non-seminomatous GCT (stages IIA/B and IIC/III), for restaging and treatment of residual masses, and for relapsed and refractory disease stages. The recommendations also cover extragonadal and sex cord/stromal tumours, the management of follow-up and toxicity, quality-of-life aspects, palliative care, and supportive therapy. Conclusion: Physicians and other medical service providers who are involved in the diagnostics, treatment, and follow-up of GCT (all stages, outpatient and inpatient care as well as rehabilitation) are the users of the present guideline. The guideline also comprises quality indicators for measuring the implementation of the guideline recommendations in routine clinical care; these data will be presented in a future publication.

Phyllodes tumours of the breast are rare fibroepithelial stromal tumours which are morphologically very different from epithelial breast cancer. Its diagnosis and management has always been challenging till the World Health Organization (WHO) divided it into two-three subtypes in 2003, only then it was found that incidence and management and follow-up of these three subtypes need to be completely different to reach the optimum outcome. We are presenting a case of a 47-year-old female who presented relatively late (due to pandemic restrictions) with a large phyllodes tumour requiring mastectomy as well as adjuvant treatment.

Until the 1990s, cytotoxic chemotherapy has been the cornerstone of medical therapy for gastrointestinal (GI) cancers. Better understanding of the cancer cell molecular biology has led to the therapeutic revolution of targeted therapies, i.e. monoclonal antibodies or small molecule inhibitors directed against proteins that are specifically overexpressed or mutated in cancer cells. These agents, being more specific to cancer cells, were expected to be less toxic than conventional cytotoxic agents. However, their effects have sometimes been disappointing, due to intrinsic or acquired resistance mechanisms, or to an activity restricted to some tumour settings, illustrating the importance of patient selection and early identification of predictive biomarkers of response to these therapies. Targeted agents have provided clinical benefit in many GI cancer types. Particularly, some GI tumours are considered chemoresistant and targeted therapies have offered a new therapeutic base for their management. Hence, somatostatin receptor-directed strategies, sorafenib, and imatinib have revolutioned the management of neuroendocrine tumours (NET), hepatocellular carcinoma (HCC), and gastrointestinal stromal tumours (GIST), respectively, and are now used as first-line treatment in many patients affected by these tumours. However, these agents face problems of resistances and identification of predictive biomarkers from imaging and/or biology. We propose a comprehensive two-part review providing a panoramic approach of the successes and failures of targeted agents in GI cancers to unravel the pharmacologic opportunities and future directions for these agents in GI oncology. In this second part, we will focus on NET, HCC, and GIST, whose treatment relies primarily on targeted therapies.

Benign surgical conditions of the oesophago-gastric junction (OGJ) are important causes of morbidity and in some cases mortality for patients. This chapter discusses both elective and emergency benign disorders of the OGJ, their investigation and management. Elective conditions include gastro-oesophageal reflux disease (GORD), giant para-oesophageal hiatal herniae (GPHH), achalasia and other motility disorders. These conditions are now usually all managed laparoscopically when operative intervention is required. Emergency conditions include acute presentations of giant para-oesophageal hiatal herniae and OGJ perforations including Boerhaave's syndrome. These conditions present diagnostic and management challenges to the surgeon and are associated with significant morbidity and mortality. Varices, gastro-intestinal stromal tumours (GISTs), ulcers and bleeding are not discussed in this article.

The 2014 WHO classifies endometrial stromal tumours into endometrial stromal nodule(ESN), low-grade endometrial stromal sarcoma (LGESS), high-grade endometrial stromalsarcoma (HGESS), and undifferentiated uterine sarcoma (UUS). LGESS and HGESS are histomorphologically, immunohistochemically and genetically distinct from each other.A 51-year-old postmenopausal lady presented to us with vaginal bleeding. Radiological findings revealed a well defined heterogeneous lesion involving the whole of uterus.Hysterectomy revealed a large polypoidal tumour, occupying the entire uterine cavity.Microscopically, the tumor was predominantly composed of epithelioid cells with very fewintervening spindle cell areas. Immunohistochemically, epithelioid cells were diffusely positive for cyclinD1, while were negative for CD10, ER, PR.Diagnosis of cyclinD1 positive HGESS was rendered. This case highlights the importance of performing cyclinD1 immunostaining in diagnosing HGESS. DOI:10.21276/APALM.1530

Endometrial stromal tumours have been recently reclassified in the WHO 2014 Classification due to the discovery of new genetic fusions. This has enabled the subdivision of previously described undifferentiated endometrial sarcomas into the molecularly-defined high grade endometrial stromal sarcoma (HG ESS) and undifferentiated uterine sarcoma (UUS). In this review, we discuss the discoveries behind the 2014 Classification and its rationale, and give practical tips for diagnosis of these neoplasms, as well as discussing the differential diagnoses that one may consider.

Objectives Ovarian cancer comprises several histologically distinct subtypes. The distribution of these subtypes varies worldwide. Survival differs between the subtypes. The CONCORD programme is the largest population-based study of global trends in cancer survival. We aimed to explore international variation in survival for each subtype, to help interpret international differences in survival from all ovarian cancers combined. Methods The third cycle of the CONCORD programme (CONCORD-3) includes data on 812,783 adult (15–99 years) women diagnosed with ovarian cancer during 2000–2014 in 61 countries. We defined six histological groups: type I epithelial, type II epithelial, germ cell, sex cord-stromal, other specific non-epithelial and non-specific morphology. Only microscopically verified tumours were included. Borderline tumours were excluded. We estimated age-standardised 5-year net survival for each country by histological group. Results Type II tumours were the commonest histological group worldwide (70%), followed by type I tumours (22%). Non-specific, other specific non-epithelial, germ cell and sex cord-stromal tumours were rare (8% of all tumours). Survival for each histologic subtype varied widely between countries. Survival from sex-cord stromal tumours was highest (80–90%). Survival ranged from 40% to 70% for type I tumours, but was much lower for type II (20–40%). Survival from germ cell tumours was generally 70–80%. Conclusions Type I, germ cell and sex cord-stromal tumours generally showed higher survival than type II tumours. The proportion of these tumours may influence survival estimates for all ovarian cancers combined. International comparisons of survival should focus on survival for each histological subtype rather than for all ovarian cancers combined.

Endometrial stromal tumours (ESTs) are rare, intriguing uterine mesenchymal neoplasms with variegated histopathological, immunohistochemical and molecular characteristics. Morphologically, ESTs resemble endometrial stromal cells in the proliferative phase of the menstrual cycle. In 1966 Norris and Taylor classified ESTs into benign and malignant categories according to the mitotic count. In the most recent classification by the WHO (2020), ESTs have been divided into four categories: Endometrial Stromal Nodules (ESNs), Low-Grade Endometrial Stromal Sarcomas (LG-ESSs), High-Grade Endometrial Stromal Sarcomas (HG-ESSs) and Undifferentiated Uterine Sarcomas (UUSs). ESNs are clinically benign. LG-ESSs are tumours of low malignant potential, often with indolent clinical behaviour, with some cases presented with a late recurrence after hysterectomy. HG-ESSs are tumours of high malignant potential with more aggressive clinical outcome. UUSs show high-grade morphological features with very aggressive clinical behavior. With the advent of molecular techniques, the morphological classification of ESTs can be integrated with molecular findings in enhanced classification of these tumours. In the future, the morphological and immunohistochemical features correlated with molecular categorisation of ESTs, will become a robust means to plan therapeutic decisions, especially in recurrences and metastatic disease. In this review, we summarise the morphological, immunohistochemical and molecular features of ESTs with particular reference to the most recent molecular findings.

This paper reviews the distribution of interstitial cells of Cajal (ICC) in the human gastro-intestinal (GI) tract, based on ultrastructural and immunohistochemical evidence. The distribution and morphology of ICC at each level of the normal GI tracts is addressed from the perspective of their functional significance. Alterations of ICC reported in as well as in GI stromal tumours are reviewed, with emphasis on the place of ICC in the pathophysiology of disease.

Background & Objectives: Prostatic Stromal Tumours of Uncertain Malignant Potential (STUMP) are non-epithelial mesenchymal spindlecell origin prostate tumours classified as a specialized stromal tumour of the prostate. They represent rare neoplastic proliferative lesions that according to WHO classification together with prostatic stromal sarcoma (SS) comprise up to 0.2% of all malignant prostate tumours. Methods: We present a case of patient clinically presented with symptoms of acute urinary retention (AUR). Digital rectal examination (DRE) showed the presence of a pronounced prostatic hyperplasia and a transurethral prostatic adenomectomy (TURPA) was performed. The primary pathological evaluation was performed at Laboratorii Uniti, Italy and second opinion at the Department of Pathology, Clinical Hospital Centre Rijeka, Croatia upon an incidental and unexpected diagnosis of prostatic STUMP. It was morphologically discriminated based on following criteria: stromal cellularity, presence of occasional mitotic figures, necrosis, and stromal overgrowth. Thorough immunohistochemistry evaluation and tumour immunophenotyping was performed. Results: Presented tumour was immunohistochemically positive for CD34, Vimentin, progesterone and cytokeratin negative. It diffusely infiltrated the prostate gland and extend into adjacent tissues. Mitotic count was low, while focally marked cellular atypia was observed throughout the lesion. The capacity of recurrence placed it into the group considered to have frank neoplastic capacity. Therefore the clinical and further therapeutic management should be made keeping in mind the probability of progression into sarcoma. Conclusion: Currently, due to a small number of cases and consequent follow up the recommended treatment algorithm does not exist. According to some authors and literature data, radical prostatectomy seem to be the treatment of choice, especially in young patient or for extensive recurrent lesion. The presented case stresses out the need for extensive sampling in order to fully appreciate tumour heterogeneity, because STUMP remains diagnosis by exclusion.

We present a small series of patients with stage I testicular sex-cord stromal tumours (TSCST), treated with organ-sparing surgery, which confirm its feasibility and safety in selected patients...

International audience; AIMS: FOXL2 mutation has been consistently identified in adult granulosa cell tumours (A-GCTs). DICER1 mutations have been described predominantly in Sertoli-Leydig cell tumours (SLCTs). The prognostic implication of these mutations remains uncertain, as moderately sized studies have yielded variable outcomes. Our aim was to determine the implications of DICER1 and FOXL2 mutations in 156 ovarian sex cord-stromal tumours (SCSTs). METHODS AND RESULTS: FOXL2 mutations were found in 94% of pathologically confirmed A-GCTs (95/101), in one of eight juvenile granulosa cell tumours (J-GCTs), and in two of 19 SLCTs. DICER1 mutations in the RNase IIIb domain were found in six of 19 SLCTs, two of eight J-GCTs, and one of 12 undifferentiated SCSTs (Und-SCSTs). Comparison of DICER1-mutated SLCTs with DICER1-non-mutated SLCTs showed that patient age at diagnosis was lower and oestrogen receptor expression was more frequent in DICER1-mutated tumours. With a median follow-up of 22 months, two of five DICER1-mutated SLCTs relapsed, in contrast to none of eight DICER1-non-mutated tumours. CONCLUSIONS: Our results suggest that, in contrast to FOXL2 mutations in A-GCT, DICER1 mutations in SLCT might be more useful for prognosis than for diagnosis. However, study of a larger cohort of patients is necessary to establish this. Identification of genetic alterations in SCST offers promising therapeutic options

Endometrial stromal tumours (EST) are rare tumours of endometrial stromal origin that account for less than 2% of all uterine tumours. Recent cytogenetic and molecular advances in this area have improved our understanding of ESTs and helped refine their classification into more meaningful categories. Accordingly, the newly released 2014 WHO classification system recognises four categories: endometrial stromal nodule (ESN), low-grade endometrial stromal sarcoma (LGESS), high-grade endometrial stromal sarcoma (HGESS) and undifferentiated uterine sarcoma (UUS). At the molecular level, these tumours may demonstrate a relatively simple karyotype with a defining chromosomal rearrangement (as in the majority of ESNs, LGESSs and YWHAE-rearranged HGESS) or demonstrate complex cytogenetic aberrations lacking specific rearrangements (as in UUSs). Herein we provide an update on this topic aimed at the practicing pathologist.

Aim: The aim of this study is to evaluate the results of a single and common approach for transgastric submucosal excision of small- to medium-sized gastrointestinal stromal tumours (GIST) having different locations in upper two-third of the stomach by pure minimally invasive approach. Materials and Methods: A retrospective study from February 2012 to January 2015 on 11 patients was conducted including both male and female. Their age ranged from 40 to 65 years. All were subjected to laparoscopic transgastric submucosal excision of GIST in upper two-third of the stomach with 1 cm margin having different locations in stomach. Excised tumours were subjected to histopathological study. These patients were followed for 12 months assessing operative time, post-operative complications, conversion rates, morbidity, hospital stay and recurrence rate. Results: Duration of surgery was 150.6 ± 20.4 min. Mean hospital stay was 8 (range: 7–10) days. There were no post-operative complications. There were no conversions to open procedure. There were no recurrences. Morbidity was comparable to any other laparoscopic procedures. Conclusion: We conclude that a single and common approach of transgastric submucosal excision of small- to medium-sized stromal tumours with 1 cm margin having different locations in upper two-third of stomach, by pure minimally invasive approach, has a better outcome and brings a uniformity in laparoscopic management of GIST.

INTRODUCTION Gastrointestinal stromal tumours (GISTs) are the most common connective tissue neoplasms of the gastrointestinal tract, the most common clinical presentation of which is with abdominal pain or gastrointestinal bleeding. METHODS We describe a case of a perforated gastric GIST as well as reviewing the relevant published literature. RESULTS A 51-year-old woman presented to the acute assessment unit with a 1-day history of severe epigastric pain on a background of longstanding reflux symptoms. Radiological investigation demonstrated a perforated mass in the gastric antrum and the patient subsequently underwent an emergency distal gastrectomy. She recovered well postoperatively and was discharged home. Her condition remains stable six months following surgery. Histological analysis revealed the perforated lesion to be a GIST. A PubMed search suggests that this is the first English report to describe a perforated gastric GIST. Six further published reports (written in English or with an English abstract) describing the presentation of small bowel GISTs with perforation are reviewed. CONCLUSIONS We present the first English report of a perforated gastric GIST. More common presentations include abdominal pain and gastrointestinal bleeding. Although rare, GISTs should be considered in the differential diagnoses of perforated gastrointestinal masses.

Aims: The aim of this study was to determine the value of morphometric features in distinguishing mild and moderate atypia and predicting the recurrence of World Health Organization 2003-defined endometrial stromal sarcoma and highly malignant undifferentiated endometrial sarcoma. Methods and results: Nuclear and cytological size, shape and arrangement were morphometrically evaluated in 41 cases with consensus no/mild (n = 38) or moderate (n = 3) atypia. None of the cases showed necrosis. The prognostic value of these features in predicting recurrence was also assessed. Seven features differed. The mean and standard deviation of the nuclear volume and the distance between the nuclei were the best discriminators between the no/mild and moderate atypia, with the maximum of the nuclear volume being a practically and rapidly evaluable alternative. With the use of these features, all mild and moderate atypias were correctly classified. Seven cases recurred. The distance between the nuclei and the percentage of nuclei with one neighbour (assessed with morphometric minimum spanning tree analysis) predicted recurrence. Conclusions: In invasive endometrial stromal tumours, morphometric features are useful diagnostic support tools for distinguishing mild from moderate atypia and predicting recurrence.

Testicular sex cord stromal tumours (SCSTs) arise from the supporting tissues of the testis. The most common clinical presentation is with a testicular mass, which most often causes swelling and/or discomfort or one of the variants of the SCSTs can be rarely present as oversecretion of testosterone or oestrogen. Eighty to ninety percent of the SCSTs are benign but their behaviour and prognosis is difficult to predict, and they cannot be differentiated from germ-cell tumours clinically or radiologically. It is very important to find features in post-orchidectomy specimens, which are associated with a malignant clinical behaviour; Kim et al. (Am J Surg Pathol 9(3):177–92, 1985) had defined the malignancy criteria. The purpose of this article is to review case series, prognostic factors, and summarise information about the site and timing of relapse to help draft recommendations for follow-up guidelines in SCSTs. Data from the literature are conflicting in terms of risk factors and optimal follow-up schedules. Pub Med was searched for keywords such as testicular SCSTs follow-up. Case studies done between 1998 and 2011 involving 146 patients were included in this review to recommend follow-up guideline for stage 1 SCSTs on the basis of risk factors.

Background Previously Gastro-Intestinal Stromal Tumours (GISTs) have been risk stratified histologically according to their size and mitotic index. However, gastric GISTs have a lower likelihood of recurrence and so the Miettinen criteria are now used to risk stratify patients. Records were reviewed from multiple centres to determine if these changes altered patients’ clinical care and also to determine the survival of patients following the introduction of imatinib therapy. Methods Prospective databases of GISTs undergoing surgical resection and those reviewed by the regional sarcoma MDT were cross-referenced and added to by searching a variety of clinical and pathology coding datasets, to identify patients diagnosed between January 2000 and March 2010. Patients undergoing resection for localised disease were re-scored using Miettinen criteria and Kaplan–Meier analysis was used to determine survival outcomes. Results The search identified 203 patients; including 132 gastric GISTs, 89 of which had resections of untreated localised disease. These were reassessed, of which approximately one third were scored as intermediate risk. Following reclassification, 26 of 29 of intermediate risk cases moved to low risk groups, representing 27.7% of all those remaining in follow-up at the time of audit. Median survival was not reached after a median follow-up of 3.85 years and 4-year survival was estimated at 72%. Conclusions Clinicians involved in the follow-up of gastric GISTs should reassess the pathology of all intermediate and high risk patients in order to decrease patient exposure to stressful interventions, as well as hospital workload, and expenditure on unnecessary observation.

Fibrosarcoma of the breast is a rare entity. Prognosis of fibrosarcoma of the breast is poor and the role of various treatment modalities is not clearly defined due to the rarity of the disease. It must be differentiated from other stromal tumours like malignant phylloid tumour, Malignant fibrous histiocytoma and pleomorphic rhabdomyosarcoma, liposarcoma, leiomyosarcoma. One such case is presented here. Primary sarcomas of the breast are extremely rare and account for less than 0.1% of all malignant tumours of the breast of which the conversion of a phyllodes tumour to sarcoma of the breast is even rarer and only two such case has ever been reported.

e23519Background: Paediatric, adolescent, wild type and syndromic Gastro Intestinal Stromal Tumours (PAWS-GIST) consortium was formed in UK as a joint effort between GIST specialists, patients and ...

Ovarian sex cord-stromal tumours are uncommon tumours and as a group comprise only approximately 8% of ovarian neoplasms, at least half of which are accounted for by the relatively commonly encountered benign fibroma. However, even less commonly encountered tumours, such as those in the granulosa and Sertoli-Leydig groups, enter into the differential of a great range of generally more common tumours. Accordingly, some reasonable awareness of the features of these uncommon tumours is crucial in overall ovarian tumour diagnosis. Although these tumours may be functioning, tumours that may mimic them may also be functioning and may present a misleading clinical background to the pathologist. This review discusses the morphology of these tumours, highlighting their characteristic and some less known morphological features, updates the reader on some newly described features such as the occurrence of pseudopapillae in some granulosa cell tumours and endometrioid-like tubules in occasional Sertoli-Leydig cell tumours, and draws attention to a newly described entity, the microcystic stromal tumour. Other issues considered in the recent literature, such as re-exploration of the topic of cellularity and mitotic activity in fibroblastic neoplasms, are also considered. Although touched upon briefly, immunohistochemistry is not considered in detail as it is reviewed elsewhere in this mini-symposium.

Surgical resection is indicated for gastrointestinal stromal tumour (GIST). Laparoscopic resection with clear margins is effective in gastric GIST. However, GIST located in the posterior gastric wall with close proximity to the oesophagogastric junction poses special challenges. Laparoscopic intragastric resection offers a new approach to the management of these tumours. The surgical technique, our experience and a summary of the literature are presented.

In the EORTC-ISG-AGITG trial 946 patients with advanced gastro-intestinal stromal tumours (GIST) were randomised to receive 400 or 800 mg of imatinib daily. An increase in progression free survival (PFS) was demonstrated for patients randomised to the high-dose arm. Patients randomised to low-dose could cross-over to high-dose upon progression. We evaluated the feasibility, safety and efficacy of this policy. Of the 241 patients available for follow-up, 133 patients (55%) crossed over to high-dose imatinib according to the protocol. Of these patients, 92% had not had a prior dose reduction. The cumulative incidence of subsequent dose reductions after cross-over was 17% after six months with 51% discontinuing therapy without requiring a dose reduction. The extent of anaemia and fatigue increased significantly after cross-over, whilst neutropenia was less severe than during low-dose treatment. Objective responses after cross-over included three patients (2%) with a partial response and 36 (27%) with stable disease. The median PFS after cross-over was 81 days, although 18.1% of patients were still alive and progression free one year after cross-over. We conclude that a cross-over to high-dose imatinib is feasible and safe in GIST patients who progress on low-dose therapy.

Aims Sex cord–stromal tumours of the testis are uncommon tumours, accounting for around 5% of testicular neoplasms. Treatment is primarily surgical, with no adjuvant therapy of proven benefit. We present a single-centre experience over a period of 15 years. Materials and methods From 1988 to 2002, 18 patients with a diagnosis of sex cord–stromal tumour were referred to our centre. A retrospective analysis of their case notes was made and a pathological review undertaken. Results Sixteen were Leydig-cell tumours and two were Sertoli cell. For the Leydig-cell tumours, the median age at presentation was 42 years, 50% presented with a testicular mass and 31% with gynaecomastia. Two patients followed a malignant course: one revealing disease dissemination at initial staging, and a second 12 months after potentially curative orchidectomy. Salvage retroperitoneal lymphadenectomy in the latter patient proved unsuccessful. Clinical outcome correlated strongly with the presence of adverse pathological features described previously in the literature. After a median follow-up of 46 months, two patients have developed progressive disease, and two patients have died, one of metastatic Leydig-cell tumour. No patient defined as being of low malignant potential on pathological examination has relapsed outside our review period of 2 years. Conclusion We confirm the overall excellent prognosis for most of the patients with sex cord–stromal tumours of the testis. Compared with most previous reports, pathological features seem to predict with reasonable accuracy the risk of malignant behaviour, and can adequately inform the subsequent review policy.

Prostatic stromal tumours are rare neoplasias that include benign, malignant and borderline lesions. Stromal tumour of uncertain malignant potential (STUMP) has been recently described and only a few reports exist in the literature. As a rare and distinct neoplasia, to date, there is no description of MRI findings of prostate STUMP. In this article, we describe the clinical and MRI features with histopathological correlation of a patient with prostate STUMP.