Clear Margins Research Articles

The Revised R Status is an Independent Predictor of Postresection Survival in Pancreatic Cancer After Neoadjuvant Treatment.

To investigate the oncological outcomes of patients with pancreatic ductal adenocarcinoma (PDAC) who had an R 0 or R 1 resection based on the revised R status (1mm) after neoadjuvant therapy (NAT). The revised R status is an independent prognostic factor in upfront-resected PDAC; however, the significance of 1mm margin clearance after NAT remains controversial. Patients undergoing pancreatectomy after NAT for PDAC were identified from 2 prospectively maintained databases. Clinicopathological and survival data were analyzed. The primary outcomes were overall survival (OS), recurrence-free survival (RFS), and pattern of recurrence in association with R 0 >1mm and R 1 ≤1mm resections. Three hundred fifty-seven patients with PDAC were included after NAT and subsequent pancreatic resection. Two hundred eight patients (58.3%) received FOLFIRINOX, 41 patients (11.5%) received gemcitabine-based regimens, and 299 individuals (83.8%) received additional radiotherapy. R 0 resections were achieved in 272 patients (76.2%) and 85 patients (23.8%) had R 1 resections. Median OS after R 0 was 41.0 months, compared with 20.6 months after R 1 resection ( P = 0.002), and even longer after additional adjuvant chemotherapy ( R 0 44.8 vs R1 20.1 months; P = 0.0032). Median RFS in the R 0 subgroup was 17.5 months versus 9.4 months in the R 1 subgroup ( P < 0.0001). R status was confirmed as an independent predictor for OS ( R 1 hazard ratio: 1.56, 95% CI: 1.07-2.26) and RFS ( R 1 hazard ratio: 1.52; 95% CI: 1.14-2.0). In addition, R 1 resections were significantly associated with local but not distant recurrence ( P < 0.0005). The revised R status is an independent predictor of postresection survival and local recurrence in PDAC after NAT. Achieving R 0 resection with a margin of at least 1mm should be a primary goal in the surgical treatment of PDAC after NAT.

Pseudoangiomatous stromal hyperplasia of the breast: Clinical evaluation.

Pseudoangiomatous stromal hyperplasia is a rare benign breast stromal proliferative lesion of the breast. Clinical presentation ranges from rapidly growing mass to incidental identification in routine screening. This difference in manifestation and its rarity makes it difficult to be a standard treatment protocol. Therefore, we aimed to share our clinical experience in Pseudoangiomatous stromal hyperplasia. The files of patients who underwent core biopsy or surgical excision due to a breast mass and resulted in pseudoangiomatous stromal hyperplasia between January 2013 and December 2021 were included in the study. 17 patients with a median age of 37 (22-68) were found Pseudoangiomatous stromal hyperplasia confirmed by surgical excision or core biopsy. Chosen treatment option was observation in 8 patients (47.1%), while surgical excision was used in 9 (52.9%) patients. The mean follow-up period was 55.24 ± 26.72 (13-102) months. None of the patients observed the Malignant transformation during the follow-up period. For Pseudoangiomatous Stromal Hyperplasia of the breast, surgical excision with clean margins or close follow-up after diagnosis confirmation by tissue biopsy is sufficient. Pseudoangiomatous Stromal Hyperplasia is not a risk factor for developing breast cancer.

Definitive Surgery after Neoadjuvant Chemotherapy for Locally Advanced Oral Cavity Cancers: Experience from a Tertiary Care Center.

Mansi AgrawalVidya KonduruBackground Oral cavity cancers require definitive surgical resection as the primary treatment, but with advanced T stage, complete resection with pathologically negative margins might be difficult to achieve. Induction chemotherapy helps achieve the balance between resection and morbidity in locally advanced technically unresectable tumors. The aim of this study was to analyze the impact of surgery in locally advanced, technically unresectable oral cavity cancers after neoadjuvant chemotherapy (NACT). Materials and Methods A retrospective analysis of patients with borderline resectable, locally advanced oral cavity cancers who were given NACT between February 2017 and December 2021 was conducted. Data regarding clinical and pathological characteristics, NACT, surgery, adjuvant therapy, and recurrences was analyzed. Results Of the 69 patients in the study, 69.6% had tongue cancer, rest were gingivobuccal complex cancers. All tumors were resected based on the post-NACT tumor volume and clear margins were achieved in 42% of cases. About 85.4% of the tongue cancers required a lesser resection than anticipated, thereby following the concept of organ and functional preservation post-NACT as proposed by Licitra et al. About 30.4% had ypT0 and 17.4% had ypN0. Recurrence and survival rates noted in our study were comparable to those reported in literature. Lymph node density of more than or equal to 0.07 was found in all recurrent cases. Conclusions Induction chemotherapy offers a chance of achieving adequate surgical resection while reducing morbidity and improving functional outcomes for patients with technically unresectable oral cavity cancers. Nodal disease may not respond to chemotherapy as well as the primary tumor. There is a need for comprehensive evaluation of prognostic factors, which could help identify the patients who will most benefit with NACT.

Clinical and functional outcome after partial or total claviculectomy without reconstruction for oncologic causes

To achieve clear margins in rare malignant clavicular neoplasms, claviculectomy may become necessary. This study aimed to review clinical, functional, and oncologic outcomes following partial or total claviculectomy without reconstruction. This study retrospectively included 15 patients from 2 tertiary sarcoma centers (mean age, 42.6±20.3 years; 66.7% male patients). The median length of clinical and oncologic follow-up was 48.0 months (interquartile range [IQR], 24.0-83.5 months). Functional follow-up (Musculoskeletal Tumor Society score and QuickDASH score [short version of the Disabilities of the Arm, Shoulder and Hand questionnaire]) was available in 9 patients at a median of 36.0 months (IQR, 20.0-100.0 months). Of the 15 patients, 7 underwent total claviculectomy; 5, partial lateral claviculectomy; and 3, partial medial claviculectomy. No postoperative complications emerged. The median Musculoskeletal Tumor Society and QuickDASH scores at latest follow-up amounted to 26.0 points (IQR, 24.0-29.0 points) and 18.0 points (IQR, 11.0-22.0 points), respectively. Notably, scores tended to be lower in patients who underwent total claviculectomy (n=2) in comparison to partial claviculectomy (n=7). Satisfactory clinical and functional results can be achieved following partial or total claviculectomy without reconstruction, with a low complication rate and acceptable mid- to long-term function.

Liver transplantation in the management of cholangiocarcinoma: Evolution and contemporary advances.

Cholangiocarcinoma (CCA) is an aggressive malignancy arising from the biliary epithelium. It may occur at any location along the biliary tree with the perihilar area being the most common. Prognosis is poor with 5-year overall survival at less than 10%, typically due to unresectable disease at presentation. Radical surgical resection with clear margins offers a chance of cure in patients with resectable tumours, but is frequently not possible due to locally advanced disease. On the other hand, orthotopic liver transplantation (LT) allows for a radical and potentially curative resection for these patients, but has been historically controversial due to the limited supply of donor grafts and previously poor outcomes. In patients with perihilar CCA, within specific criteria and following the implementation of a protocol combining neoadjuvant chemoradiation and LT, excellent results have been achieved in the last decades, resulting in its increasing acceptance as an indication for LT and the standard of care in several centres with significant experience. However, in intrahepatic CCA, the role of LT remains controversial and owing to dismal previous results it is not an accepted indication. Nevertheless, more recent studies have demonstrated favourable results with LT in early intrahepatic CCA, indicating that, under defined criteria, its role may increase in the future. This review highlights the history and contemporary advances of LT in CCA, with particular focus on the improving outcomes of LT in intrahepatic and perihilar CCA and future perspectives.

Predictors of recurrence in oral cavity cancer with clear surgical margins

Introduction: Oral cancers recur in a significant proportion of patients, in spite of aggressive treatment strategies. The presence of a clear surgical margin is an important predictor of recurrent disease, among others. Since oral cancers often recur in the absence of compromised margins, there is a need to study the factors affecting recurrence and overall survival outcomes where clear surgical margins have been achieved during upfront surgery. This study attempts to identify the significant predictors of locoregional recurrence in oral squamous cell carcinoma (OSCC) with pathologically clear surgical margins. Methodology: This retrospective study was done to study the clinicopathological parameters associated with recurrence of oral cavity squamous cell carcinoma (SCC) in patients with clear surgical margins operated in our unit between January 2010 and December 2015. A total of 526 cases of oral cavity SCC were analyzed and records of 160 cases with clear surgical margins were reviewed for clinical details, histopathological data, and follow-up status. Age, gender, subsite, T–N clinical and pathological staging, tumor depth of invasion, grade of differentiation, lymphovascular invasion, perineural spread, adjuvant therapy, and recurrence details were analyzed. Results: Lymphovascular Invasion was found to be a significant predictor for local recurrence in OSCC in both univariate and multivariate analysis. Median recurrence-free survival was 53.6 months. Conclusion: Despite the best efforts of the surgeon in obtaining adequate tumor-free margins and the most comprehensive adjuvant treatment, recurrence patterns in oral cancers continue to defy accurate prediction. Lymphovascular invasion could be an important predictor of recurrence for oral cavity cancers in patients with clear surgical margins that require aggressive management.

Dermatofibrosarcoma protuberans of the scalp: Surgical management in a multicentric series of 11 cases and systematic review of the literature

BackgroundDermatofibrosarcoma protuberans is a rare, slow-growing soft-tissue malignancy originating in the dermis that is characterized by an infiltrating growth pattern with a marked tendency of local recurrence. Complete surgical resection with pathological margin clearance must be achieved to reduce the risk of tumor recurrence. Resulting defects often require extensive reconstructive procedures. Dermatofibrosarcoma protuberans of the scalp poses particular challenges owing to the proximity to the face and brain. This study aims to evaluate treatment options and proposes an algorithm for management of scalp dermatofibrosarcoma protuberans based on a multicentric case series and systematic review of the literature. MethodsA retrospective multicentric chart analysis of 11 patients with scalp dermatofibrosarcoma protuberans who presented within the last 20 years was performed regarding demographic data, pathological tumor characteristics, and surgical management (resection and reconstruction). Additionally, a further 42 patients (44 cases) were identified through a systematic Preferred Reporting Systems for Systematic Reviews and Meta-Analysis–based review of the literature searching the Medline and Embase databases. ResultsIn total, 30 cases were classified as primary and 20 cases as recurring scalp dermatofibrosarcoma protuberans (data from 5 cases were missing). The median tumor size was 24 cm2 (interquartile range 7.8–64), and the median defect size was 55.8 cm2 (interquartile range 48–112). Recurring scalp dermatofibrosarcoma protuberans was more often associated with invasion of deeper layers and required more extensive tumor resection to achieve negative margins. Within the subgroup that was managed with peripheral and deep en face margin assessment, no recurrence was observed. Most patients required local (41. 8%) or free flap (27.8%) reconstruction after dermatofibrosarcoma protuberans resection. ConclusionWhenever possible, peripheral and deep en face margin assessment–based techniques should be preferred for resection of scalp dermatofibrosarcoma protuberans because they provide superior oncological safety while preserving uninvolved tissue. Patients with locally advanced and recurring scalp dermatofibrosarcoma protuberans often require multidisciplinary treatment including neurosurgery, radiotherapy, and microvascular reconstructive surgery and should be referred to a specialized center.

Simple recommendation for cases requiring re-excision on breast surgery

Breast cancer is an important health problem that especially affects women. In breast cancer where surgical procedures are the primary treatment modality, breast-conserving surgery with radiotherapy is becoming more popular than mastectomy. Re-excision may be necessary in cases of recurrence or positive surgical margins. Damage to the cavity formed due to scar formation in the old operation lodge may make dissection difficult. Failure to see the dissection plan clearly may result in incomplete surgery or removal of excess tissue. We present how we macroscopically removed the lesion with clear margins with a simple technique we performed during surgery in a patient who underwent breast-conserving surgery for breast cancer and presented with suspicion of recurrence.

Desmoid-type fibromatosis of transverse mesocolon

Desmoid tumors represent a rare disease. The recommendations for treatment and approach of these neoplasms are sparse. There are no specific imaging or clinical features which turns the diagnosis difficult. A 53-year-old male patient presented with complaints of peri-umbilical abdominal pain associated with a palpable mass. CT scan revealed a heterogeneous mass with soft tissue density and well-defined contour with approximately 8.5×7×5.3 cm localized in the mesogastric region with apparent origin in the transverse colon. The purposed etiology was of GIST. Colonoscopy was normal. The patient was submitted to surgery which revealed a mass with bright and smooth surface that was in relation with the mid portion of the transverse colon, we performed a right extended hemicolectomy. The postoperative was uneventful. Pathology evaluation revealed a desmoid-type fibromatosis. The accurate etiology of desmoid tumors is still unknown. Desmoid tumors are rare with an annual incidence of 2-4 cases per million people. They typically are slow growing fibrous soft tissue tumors with a benign behavior, however show local aggressiveness with a high ability for recurrence, complete surgical resection with clear margins is crucial.

Prognostic significance of margin clearance in pancreaticoduodenectomy specimens with pancreatic ductal adenocarcinoma in a Danish population-based nationwide study

BackgroundIn this nationwide population-based cohort study, we investigated the overall minimum margin width that is independently associated with improved survival following pancreaticoduodenectomy (PD) for pancreatic ductal adenocarcinoma (PDAC) and evaluated whether certain margins or surfaces hold independent prognostic significance. MethodsData from 367 patients who underwent PD for PDAC in the period 2015–2019 were retrieved from the Danish Pancreatic Cancer Database. Missing data were obtained by review of pathology reports and re-microscopy of resection specimens. Surgical specimens were evaluated using a standardised pathological protocol involving multicolour inking, axial slicing and exact reporting of circumferential margin clearances in 0.5 mm increments. ResultsWhen categorised according to margin widths of <0.5, <1.0, <1.5, <2.0, <2.5 and <3.0 mm, R1 resections were detected in 34%, 57%, 75%, 78%, 86% and 87% of cases, respectively. In multivariable analyses, an overall margin clearance of ≥1.5 mm was associated with improved survival compared with a clearance of <1.5 mm (HR 0.70 95% CI 0.51–0.97 (p = 0.031)). When evaluating the margins separately, no margin had independent prognostic significance. ConclusionMargin clearance of at least 1.5 mm was independently associated with improved survival following PD for PDAC.

Similar local recurrence and survival in patients with T1 radial growth phase melanoma on head and neck treated with 5 or 10 mm margins: A retrospective study.

Melanoma guidelines recommend surgical excision with 10 mm margins for T1 melanomas (invasive melanomas with Breslow thickness ≤1 mm), including those in radial growth phase, which are without metastatic potential; however, such margins may be problematic on head-and-neck. We compared outcomes of wide (10 mm margins) versus narrow (5 mm margins) excisions in patients with radial growth phase T1 melanoma on head-and-neck including face. We retrospectively examined 610 consecutive patients excised with wide versus narrow margins, from 2001 to 2018, at six European centres. In all cases, radial growth phase, and clear margins with 5 or 10 mm of clearance, were ascertained histologically. Multivariable models investigated associations of margins and other factors with overall survival and local recurrence. Three hundred and sixteen (51.8%) patients received wide excision, 219 (69.3%) with primary wound closure, 97 (30.7%) with reconstruction; 294 (48.2%) patients received narrow excision, 264 (89.8%) with primary wound closure, 30 (10.2%) with reconstruction (p < 0.001). Median follow-ups were 88 months (wide) and 187 months (narrow) (inter-quartile ranges 43-133 and 79-206, respectively). Ten-year overall survival (95% confidence interval) was 96.7% (94.2%-99.3%) in wide and 98.2% (96.4%-100%) in narrow patients. Ten-year local recurrence incidence was 6.4% (4.1%-10.1%) in wide and 7.8% (5.3%-11.6%) in narrow groups. Lentigo maligna melanoma subtype appeared associated with increased risk of local recurrence in narrow versus wide patients (15.0% vs. 7.5%; p=0.190). Narrower excision margins for T1 radial growth phase melanoma are not associated with worse overall survival (hazard ratio 0.97, p=0.996) or increased local recurrence (subdistribution hazard ratio: 0.87; p=0.751) compared to wider margins, and may be safely applied to such lesions, although caution may be required in the presence of lentigo maligna melanoma.

Risk factors affecting benign anastomotic stricture in anterior and low anterior resections for colorectal cancer: a single-center retrospective cohort study.

Although not as life-threatening as anastomotic leakage, anastomotic stricture reduces the quality of life. The risk factors for such an important life complication have not been revealed. This article examines the risk factors affecting anastomotic strictures due to colorectal cancers. Patients who underwent anterior and low anterior resection for colorectal cancer under elective conditions between 2015 and 2021 were included in the study. The patients were divided into two groups, those who developed anastomotic stricture and those who did not. The parameters determined between the two groups were compared, and multivariate analysis of statistically significant parameters was performed. A total of 375 patients were included in the study. The anastomotic stricture was detected in 36 (9.6%) patients. In the multivariate analysis, non-mobilization of the splenic flexure and a proximal clean surgical margin of < 10cm and a distal surgical margin of < 2cm were identified as risk factors affecting anastomotic stricture. The risk factor with the highest odds ratio in the development of anastomotic stricture is the non-mobilization of the splenic flexure (p = 0.001, OR 11.375). It is recommended that the mobilization of the splenic flexure to reduce the development of strictures. In addition, a clean surgical margin of 10cm proximally and 2cm distally and high ligation of the inferior mesenteric artery may reduce the development of stricture.

The usefulness of intraoperative sodium fluorescein in the surgical treatment of relapsed high-grade brain tumors in pediatric patients.

We report the usefulness of intraoperative sodium fluorescein (SF) in the surgical treatment of relapsed high-grade brain tumors in pediatric neurosurgery. We describe our protocol for intraoperative SF and three cases of patients between 5 and 11years diagnosed and surgically treated for relapsed high-grade brain tumors using SF. The 560-nm microscope filter enables the use of low doses of this fluorochrome. A dose of 3mg/kg of patient weight of 10% SF, administered intravenously, is safe and effective in children. The effect of SF was immediate, providing a clear margin between the tumor and healthy tissue, which enabled good tumor resection. We observed no adverse effects in the postoperative period, and the patients evolved satisfactorily. To the best of our knowledge, we describe for the first time the use of fluorescein in reoperations of relapsed high-grade brain tumors in childhood with promising results. Using SF in children is a safe, affordable, and effective technique that offers an excellent intraoperative image, being a feasible option to improve oncological resection. This study is one of the few that uses SF in pediatric neurosurgery, where it could be very beneficial.

Fibromatosis of the breast; another diagnosis of cancer like lesions: About 4 cases reports

Introduction and importanceDesmoid fibromatosis is a benign monoclonal fibroblastic tumor that was first reported in 1832 and arises from musculoaponeurotic structures. It is an uncommon tumor that can mimic breast carcinoma in both hits clinical and radiological presentation.This entity mainly affects patients in their fourth and fifth decades of life. Case presentationOur four patients were of young women in their 2nd and 3rd decade that consulted for masses of the breast.These lesions were suspicious for malignancy in both their clinical and radiological features despite not having any family history of breast carcinoma. Clinical discussionOnly histology provides the diagnosis, it is locally invasive, non-metastatic but with a high potential of recurrence and the sole treatment is complete and wide local excision with clear margins. ConclusionAlthough it is a rare clinical scenario, the presence of an unusual breast or chest wall tumor such as a desmoid tumor should be considered when obvious breast changes and history do not correlate with routine diagnostic measures.

Emerging technology for intraoperative margin assessment and post-operative tissue diagnosis for breast-conserving surgery

IntroductionTissue-preserving surgery is utilized progressively in cancer therapy, where a clear surgical margin is critical to avoid cancer recurrence, specifically in breast cancer (BC) surgery. The Intraoperative pathologic approaches that rely on tissue segmenting and staining have been recognized as the ground truth for BC diagnosis. Nevertheless, these methods are constrained by its complication and timewasting for tissue preparation. ObjectiveWe present a non-invasive optical imaging system incorporating a hyperspectral (HS) camera to discriminate between cancerous and non-cancerous tissues in ex-vivo breast specimens, which could be an intraoperative diagnostic technique to aid surgeons during surgery and later a valuable tool to assist pathologists. MethodsWe have established a hyperspectral Imaging (HSI) system comprising a push-broom HS camera at wavelength 380∼1050 nm with source light 390∼980 nm. We have measured the investigated samples' diffuse reflectance (Rd), fixed on slides from 30 distinct patients incorporating mutually normal and ductal carcinoma tissue. The samples were divided into two groups, stained tissues during the surgery (control group) and unstained samples (test group), both captured with the HSI system in the visible and near-infrared (VIS-NIR) range. Then, to address the problem of the spectral nonuniformity of the illumination device and the influence of the dark current, the radiance data were normalized to yield the radiance of the specimen and neutralize the intensity effect to focus on the spectral reflectance shift for each tissue. The selection of the threshold window from the measured Rd is carried out by exploiting the statistical analysis by calculating each region's mean and standard deviation. Afterward, we selected the optimum spectral images from the HS data cube to apply a custom K-means algorithm and contour delineation to identify the regular districts from the BC regions. ResultsWe noticed that the measured spectral Rd for the malignant tissues of the investigated case studies versus the reference source light varies regarding the cancer stage, as sometimes the Rd is higher for the tumor or vice versa for the normal tissue. Later, from the analysis of the whole samples, we found that the most appropriate wavelength for the BC tissues was 447 nm, which was highly reflected versus the normal tissue. However, the most convenient one for the normal tissue was at 545 nm with high reflection versus the BC tissue. Finally, we implement a moving average filter for noise reduction and a custom K-means clustering algorithm on the selected two spectral images (447, 551 nm) to identify the various regions and effectively-identified spectral tissue variations with a sensitivity of 98.95%, and specificity of 98.44%. A pathologist later confirmed these outcomes as the ground truth for the tissue sample investigations. ConclusionsThe proposed system could help the surgeon and the pathologist identify the cancerous tissue margins from the non-cancerous tissue with a non-invasive, rapid, and minimum time method achieving high sensitivity up to 98.95%.

Autofluorescence-Raman Spectroscopy for Ex Vivo Mapping Colorectal Liver Metastases and Liver Tissue

IntroductionIdentifying colorectal liver metastases (CRLM) during liver resection could assist in achieving clear surgical margins, which is an important prognostic variable for both disease-free and overall survival. The aim of this study was to investigate the effect of auto-fluorescence (AF) and Raman spectroscopy for ex vivo label-free discrimination of CRLMs from normal liver tissue. Secondary aims include exploring options for multimodal AF-Raman integration with respect to diagnosis accuracy and imaging speed on human liver tissue and CRLM. MethodsLiver samples were obtained from patients undergoing liver surgery for CRLM who provided informed consent (15 patients were recruited). AF and Raman spectroscopy was performed on CRLM and normal liver tissue samples and then compared to histology. ResultsAF emission spectra demonstrated that the 671 nm and 775/785 nm excitation wavelengths provided the highest contrast, as normal liver tissue elicited on average around eight-fold higher AF intensity compared to CRLM. The use of the 785 nm wavelength had the advantage of enabling Raman spectroscopy measurements from CRLM regions, allowing discrimination of CRLM from regions of normal liver tissue eliciting unusual low AF intensity, preventing misclassification. Proof-of-concept experiments using small pieces of CRLM samples covered by large normal liver tissue demonstrated the feasibility of a dual-modality AF-Raman for detection of positive margins within few minutes. ConclusionsAF imaging and Raman spectroscopy can discriminate CRLM from normal liver tissue in an ex vivo setting. These results suggest the potential for developing integrated multimodal AF-Raman imaging techniques for intraoperative assessment of surgical margins.

Vulvar Paget’s Disease: A Systematic Review of the MITO Rare Cancer Group

Vulvar Paget's disease (VPD) is a rare form of cutaneous adenocarcinoma of the vulva, which accounts for about 1-2% of all vulvar neoplasms and mainly affects post-menopausal women. The clinical presentation is usually non-specific and mimics chronic erythematous skin lesions; therefore, the diagnosis is often difficult and delayed. Although VPD is typically diagnosed at a locally advanced stage and has a high recurrence rate, the prognosis is overall favorable with a 5-year survival of nearly 90%. Due to the limited and poor-quality evidence, there is no global consensus on optimal management. Therefore, we performed a systematic review of the literature through the main electronic databases to deepen the current knowledge of this rare disease and discuss the available treatment strategies. Wide surgical excision is recommended as the standard-of-care treatment and should be tailored to the tumor position/extension and the patient's performance status. The goal is to completely remove the tumor and achieve clear margins, thus reducing the rate of local recurrences. Non-surgical treatments, such as radiotherapy, chemotherapy, and topical approaches, can be considered, especially in the case of unresectable and recurrent disease. In the absence of clear recommendations, the decision-making process should be individualized, also considering the new emerging molecular targets, such as HER2 and PD-L1, which might pave the way for future targeted therapies. The current review aims to raise awareness of this rare disease and encourage international collaboration to collect larger-scale, high-quality evidence and standardize treatment.

Cytodiagnosis of multilobulated aggressive angiomyxoma clinically mimicking sarcoma – A case report

: Aggressive angiomyxoma is a rare but locally aggressive infiltrative mesenchymal tumour arising in the soft tissues of the lower genital tract. Incidence is very less (only about 350 cases have been recorded in the literature) with female to male ratio of 6:1. Average age at presentation is around 40 years.: A 32 years old female presented with multiple swelling in the vulval region for 1 and a half years associated with mild pain. The patient complains of dysmenorrhoea, pressure sensation, pelvic fullness, dyspepsia, weight loss and generalised weakness. She is married and has a 3-year old male child. Her menstrual cycle is regular with normal menses. FNAC of the vulval swelling was suggestive of angiomyxoma. The swelling was excised in the Gynaecological Operation Theatre and the specimen was sent to the Department of Pathology for Histopathological examination where it was diagnosed as aggressive angiomyxoma. On follow up, the patient was healthy with no complains of recurrence so far. : Aggressive angiomyxoma is an aggressive tumour because it has a high risk of infiltration and local recurrence. Therefore, it should be distinguished from other myxoid tumours. Preoperative cytological diagnosis helps in surgical management with appropriate wide local excision with clear margins.

Giant retroperitoneal dedifferentiated liposarcoma - A rare case report

Retroperitoneal liposarcomas are uncommon malignancy accounting for 0.15% of all malignancies. The overall survival and prognosis is predominantly based on the total completeness of resection i.e. margin status and the histopathologic grade of the tumour. The retroperitoneal sarcomas usually present late in the disease course and often develops local invasion of the vital structures at the time of clinical presentation which makes resection with clear margins difficult and challenging. Here we present a case of Giant dedifferentiated liposarcoma arising from retroperitoneum.

Our experience with solitary fibrous tumors in the chest area.

The article reports on three patients with a solitary fibrous tumor of the chest. The first patient had a tumor in the area of the dome of the right pleural cavity which was radically resected together with the chest wall around its origin. In the second case, the tumor was attached by a vascular pedicle to the lower lobe of the right lung. This tumor was resected atypically, via thoracotomy, along with a margin of healthy lung tissue at the base of its pedicle. The last patient had a tumor of the lower lobe of the right lung, surrounding the lower pulmonary vein, which did not have a clear margin of healthy lung tissue. This finding required right lower lobectomy via posterolateral thoracotomy. The presented cases describe rare types of tumors in the chest area which at the time of detection often reach large dimensions, necessitating extensive surgical procedures. Due to the biological nature of these tumors, long-term patient follow-up is advisable.