Female urethral diverticular cancer is a very rare entity with only around 100 cases reported so far in literature and accounts for <1% of all malignancies. In this article, we present a 47-year-old African American female with repeated hospital visits for urinary retention, hematuria, and urinary tract infections. Initial computed tomography imaging and cystoscopy was unremarkable except for a distended urinary bladder. Subsequent magnetic resonance imaging and corresponding cystoscopy eventually indicated the presence of a urethral diverticulum. She underwent urethral diverticulectomy and was found to have a mass arising from urethral diverticulum extending to vaginal walls. Her biopsy was suggestive of invasive adenocarcinoma in advanced stages, for which she subsequently underwent a total pelvic exenteration. Next-generation sequencing of the tumor showed CDKN2A/B loss, MSI-stable, and low TMB, thereby ruling out the options for targeted therapies. Extensive literature search and expert opinions were sought for her case since no consensus exists regarding the optimal therapeutic approach due to the rarity of this tumor. A final decision was made to treat her with platinum-based chemotherapy. Different treatment approaches including neoadjuvant chemoradiation followed by surgery, surgery followed by adjuvant chemotherapy, and surgery followed by chemoradiation have been tried. Platinum-based chemotherapy has generally been preferred based on an extensive literature search. Multimodality treatment approach seems to be the current approach to management for advanced stages for better overall survival. This case illustrates the challenges faced in making diagnosis and treatment decisions due to the rarity of this type of tumor and lack of consensus in the treatment approach. The prevalence of urethral diverticula is about 1% to 6% in adult women with most exhibiting only benign features24 even making urethral diverticular malignancies rarer.The incidence of primary urethral cancer is estimated to be 4.3 per million in males and 1.5 per million in females in a study of roughly 10% of the US population,5 As of 1992, there were only a total of 59 cases reported in the English literature,6 and by 2009, there were about 76 cases reported.7 Most recent studies suggest that about 200 cases may have been reported thus far.2 The lack of established treatment strategies partly due to the few numbers of reported cases necessitates further study on the history and the evolution of the management for this very rare malignancy. We hope to highlight the various diagnostic and treatment modalities on the backdrop of the advances in the management of cancers today, particularly with the advent of targeted therapy. This report also aims to reiterate a simpler point of ruling out the possibility of malignant lesions in patients with urinary retention/obstruction especially in the lower urinary tract and should prompt thorough physical examination with palpation of the anterior vaginal wall and appropriate imaging.The earliest case of urethral diverticular carcinoma was reported in 1952.This is the case of a 47-year-old female with medical history only remarkable for hypertension and asthma who first presented in November 2016 with urinary symptoms. She presented with hematuria and urinary frequency and was treated for a urinary tract infections with antibiotics. Subsequently, she continued to have urinary frequency, straining, and complete inability to void. She presented again in January 2017 with hematuria and urinary retention. Urology consult was obtained after staff encountered difficulty inserting a Foley catheter. The catheter was eventually inserted with a lot of resistance. Computed tomography (CT) scan done on the same visit was remarkable for a markedly distended bladder without evidence of obstruction by stone or evidence of hydronephrosis. She was scheduled for a urology clinic visit the following day where she was seen and instructed to remove the Foley catheter the day after. She returned to the emergency department after she removed the Foley catheter as instructed and was unable to urinate for up to 6 hours.Cystoscopy done during multiple visits and magnetic resonance imaging evaluation eventually revealed the presence of periurethral cysts with a diagnosis of urethral diverticula prompting a urethral diverticulectomy. Biopsy results from samples taken during the diverticulectomy revealed an invasive adenocarcinoma. Follow-up cystoscopy did not show the ostium of the diverticulum but showed a friable mass that was adherent to the vaginal wall. CT scan of the chest, abdomen, and pelvis did not show any disease burden above the diaphragm. She did not have any evidence of bone metastasis during that presentation.The patient underwent surgical resection of the mass with cystourethrectomy, anterior vaginectomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, bilateral pelvic lymph node dissection, appendectomy, a continent catheterizable reservoir (Indiana pouch) formation with primary ileocolonic re-anastomosis, and bilateral ureteral catheterization with a post-surgery diagnosis of T4N1MX stage 4 urethral diverticular adenocarcinoma. Margins post-surgery were found to be uninvolved with the adenocarcinoma.Next-generation sequencing of tissue samples results showed CDKN2A/B loss, MSI-stable, TMB (tumor mutation burden)-low, and variants in the following genes: ATM, BARD1, FAT1, FLT4, KEAP1, MLL3, PIK3, and TAF1. Treatment with platinum-based regimens was decided based on extensive literature review and expert opinion and after genomic sequencing results showed no clear role of targeted therapy. A decision was made to treat her with chemotherapy combining platinum with 5-FU (fluorouracil), which has shown to improve progression-free survival when compared with other combination regimens. The patient has now received 5 cycles of cisplatin, 5-FU with leucovorin, and treatment has been well tolerated with no clinical signs of recurrence.She continues to be followed by Urology and Oncology. Further need for concurrent radiotherapy was considered; however, the patient eventually refused.810 In our case, suspicion of a mass arose with the difficult catheterization. After identifying these lesions, a multimodality therapeutic strategy including surgery, radiation, and chemotherapy is often indicated for definitive management.9 Multimodality therapy offers more benefit especially when the disease is advanced. Tissue diagnosis often following surgery usually highlights the nature of the disease. Adenocarcinomas, transitional- and squamous-type histology are the most commonly occurring, with the majority being adenocarcinoma.7 Cases of papillary urothelial carcinoma and papillary adenocarcinoma of the urethral diverticula were also reported.12 Further differentiation of the adenocarcinomatous types is seen, for example, in cases of clear cell and signet cell (a type of mucinous) adenocarcinoma.1316 Of the 84 cases reviewed in this article, 64% were adenocarcinomas, 21% were transitional cell types, and 14% were squamous cell. The major prognostic factors for these types of lesions include the local extent of the tumor and the histologic characteristics, lymph node status, with some reports concluding that tumor size and histology were independent prognostic factors for survival and local tumor control.17 Another study reported that tumor stage, nodal status, and site of disease were independent predictors of survival.18 There is still a difference of opinion about the histologic types with the worst prognosis17; however, latest studies regarding chemotherapy and particularly targeted therapy are less focused on tumor histology, rather on the staging of the disease.19 There is also evidence that neoadjuvant chemotherapy with surgery offers better outcomes than surgery followed by adjuvant chemotherapy alone,20 and both these treatment plans involve platinum-based therapy as a common option for chemotherapy.As in other malignancies, the diagnosis of suspected urethral malignancies often includes a combination of CT/magnetic resonance imaging and endoscopic procedures (cystoscopy), aptly indicated in patients with suspected lesions obstructing urinary outflow with a classic collection of symptoms including urinary incontinence, irritative voiding symptoms, hematuria, recurrent urinary tract infection, and a palpable anterior vaginal/pelvic mass.2123 These lesions share similarities with other adenocarcinomas; hence, the suggestion of chemotherapy regimens such as platinum-based therapy like the 5-FU, leucovorin, and cisplatin combination are commonly used in colonic adenocarcinomas.25 Several other cancers especially those of genitourinary origins such as bladder cancer and primary urethral cancers are also known to have similar chemotherapeutic treatments as urethral diverticular cancers based on extrapolated evidence, hence the role of various other chemotherapeutic combinations that have been shown to have benefits, for example, gemcitabine/cisplatin (GC) combination and methotrexate/doxorubicin/vincristine/cisplatin (MVAC) combination.26 The difference in histologic types has also been seen to vary based on what part of the diverticular the tumor originates from and based on immunoreactivity with carcinoembryonic antigen and prostate-specific antigen.22The adenocarcinoma of urethral diverticulum given its glandular origin is suspected to have arisen from the paraurethral glands as some studies have highlighted its staining properties with carcinoembryonic antigen.17 The first example of multimodal therapy involving surgery, radiation, and chemotherapy was in 200310 using cisplatin, 5-FU, and leucovorin. The treatment plan was also largely based on the stage of the disease, but it is important to note that patients often are diagnosed in the advanced stages of the disease.Before the use of chemotherapy for these tumors, most treatment plans involved surgery , radiation therapy alone, and surgery and radiation combined.We will now further examine the role of various treatment modalities including targeted therapy in the management of these urethral diverticular malignancies. Rare cases as this one gives us another opportunity to reexamine the evolution of cancer therapy and shine a light on how far we have come and the very long journey ahead.8 A combination of surgery and radiation was studied showing a 44% recurrence with just diverticulectomy and/or radiation.9 Another study showed that the 5-year overall survival was 42%, and the 5-year cause-specific survival was 40% with aggressive treatment with surgery and radiotherapy.17 Aggressive treatment with surgery and radiation, albeit quite popular. is however not without a significant degree of complications.17 A more recent report shows a case of lymphatic disease progression 1 year after treatment with surgery and chemotherapy.13 Other reports, even more recent, show no recurrence at 12 months after being treated with surgery alone.27 It is important to note that these cases varied based on the stage, as less advanced stages were more amenable to surgery and radiation alone.Historically, management of urethral lesions has involved surgery with a subsequent addition of radiation with or without surgery in some cases.2830 It is important here to note that some cancers may be platinum refractory, and proper identification of treatment options following a failure in platinum-refractory cases should be decided. The role of chemotherapy also varies whether it is used as adjuvant or neoadjuvant with or without radiation.20 Chemotherapy used as neoadjuvant with or without radiation has been shown to be superior to the adjuvant options.20 Commonly used platinum-based chemotherapy options include the 5-FU/leucovorin/cisplatin,10 GC,29 MVAC,26 and paclitaxel/ifosfamide/cisplatin30 with the only comparison between GC and MVAC showing benefits to similar degrees.26 GC or MVAC combinations may be used in patients with previous sensitivity to platinum-based therapy. Other taxane options 19 also serve as an alternative therapy in patients with platinum-refractory cases. Irinotecan-based combinations reportedly show only a modest response as adjuvant regimens.31Several studies reported benefits in patients treated with platinum-based therapy for urogenital malignancies including the urethral diverticular carcinoma.10 mostly as neoadjuvant20 to shrink tumor size before surgery. Another report showed a case of a T4N0M0 primary urethral tumor treated with neoadjuvant paclitaxel, ifosfamide, and cisplatin combination followed by radiation with synchronous weekly cisplatin, and the patient was relapse-free 48 months after diagnosis, with normal voiding and sexual function.30A good number of the studies we examined employed the use of chemotherapy in combination with radiation, and the first as mentioned earlier was in 200333 and taxanes such as docetaxel although the latter is yet to be studied in a phase III trial but still believed to be a viable alternative as a salvage therapy.19 It is also important to note that patients who fail platinum-based therapy generally have poor prognosis.33 In another study,34 dramatic response was seen in a patient after treatment with nanoparticle albumin-bound paclitaxel after failure of ifosfamide/paclitaxel/cisplatin and irinotecan/5-FU/leucovorin combinations. For patients who have previously shown sensitivity to platinum-based therapy, the recommended combinations include the GC or the MVAC.Treatment recommendations for patients who have failed platinum-based therapy include vinflunine33 It is important to note that patients in the aforementioned study were selected irrespective of the PD1 or PDL1 expression, as the common criteria for selection were the disease stages and history of failure of platinum-based therapy, ultimately suggesting targeted therapy as a viable alternative when all else fails. A phase II trial is currently ongoing at the time of this report on the use of palbociclib, a CDK inhibitor, for metastatic urothelial cancer after failure of first-line chemotherapy; however, our patient could not be enrolled because genetic testing showed a loss in the CDK gene instead of an amplification. Options such as nivolumab combined with ipilimumab have shown benefits with cases of higher tumor mutational burden, but unfortunately not used in our patient as the TMB was low.35 On a final note, it is imperative that tissue diagnosis and treatment planning should include genomic sequencing to identify clearer roles for otherwise unmentioned targeted therapy options.The role of targeted therapy often comes into focus after patients have failed the initial platinum-based therapy. With the advent of PDL1 and PD1 based options such as atezolizumab, nivolumab, durvalumab, and pembrolizumab, and their use in wide variety of cancers, there is the opinion that urogenital cancers may also benefit from these new therapies. The KEYNOTE-045 trial examined 542 patients with advanced urothelial cancer that recurred or progressed after platinum-based chemotherapy and assigned them randomly to receive pembrolizumab versus investigator’s choice of chemotherapy with paclitaxel, docetaxel, or vinflunine. The pembrolizumab group showed a modest 3-month extension in overall survival compared with therapy with paclitaxel, docetaxel, or vinflunine with a lower rate of treatment-related adverse events.Due to the paucity of information on urethral diverticular carcinomas, management of these cases can often be challenging. We, therefore, encourage prompt reporting of rare cases like this as it only further advances our understanding, thus allowing newer perspectives in management.Click here for additional data file.Supplemental material, Supplementary_information for A Rare Case of Advanced Urethral Diverticular Adenocarcinoma and a Review of Treatment Modalities by Fatai Oluyadi, Preethi Ramachandran and Vladmir Gotlieb in Journal of Investigative Medicine High Impact Case Reports


Large bowel obstruction (LBO) warrants prompt evaluation and management. Although causes of LBO are most commonly intrinsic to the colon , rare extrinsic etiologies exist. An extremely rare extrinsic etiology of LBO described only once, is compressive splenic hematoma.A 64-year-old female presented to the emergency department complaining of two days of diffuse abdominal pain and distension, watery diarrhea and nausea subsequent to a mechanical fall to her left side.Computed tomography demonstrated a grade 3 splenic hematoma with active extravasation, causing extrinsic compression and obstruction of the colon. Embolization of the splenic artery was performed, and non-operative LBO management resulted in resumption of normal bowel function after six days.To our knowledge, the only other case of colonic compression by splenic hematoma (a case report in the radiology literature from 1994) describes a 62-year-old male whose symptoms similarly spontaneously resolved. Increasing frequency of non-operative management of splenic trauma may result in increased frequency of splenic hematoma complications. Physicians and surgeons who treat LBO should be aware of this rare etiology and its potential for non-operative management.Our case demonstrates the importance of considering splenic hematoma as an etiology of LBO, particularly in the setting of trauma and that management of this entity can be successfully non-operatively. Less coPrompt evaluation and management of LBO are essential, and delays may result in poor outcomes, including dehydration, critical electrolyte aberrancies, bowel perforation and death . Urgent Management of splenic trauma is increasingly non-operative , and a unique array of complications may result from splenic SNOM . GastroiHerein, we present a case of extrinsic LBO caused by splenic hematoma after a fall. To our knowledge, this is only the second such case described. This work has been reported in line with the SCARE criteria .2A 64-year-old female with past medical history of hemorrhagic stroke status-post ventriculo- peritoneal shunt and brain aneurysm clipping, seizures and multiple episodes of small bowel obstructions and exploratory laparotomies with lysis of adhesions presented by ambulance to the emergency department of an urban university-affiliated community-based trauma center, complaining of diffuse abdominal pain, distension and pressure for two days. She endorsed having watery diarrhea and nausea and no flatus for one day but denied other systemic complaints. She ambulates with an assistive device and had a fall to her left side in proximity to the start of her abdominal symptoms.On physical exam, her abdomen was tender diffusely but non-peritoneal and profoundly distended with hypoactive bowel sounds and increased tympany.Computed tomography (CT) of the abdomen and pelvis demonstrated a grade 3 splenic hematoma with evidence of active extravasation and intra-abdominal fluid consistent with hemorrhage. The CT also revealed extrinsic colonic compression by the splenic hematoma which was causing a large bowel obstruction (LBO)—the obstruction had decompressed through the incompetent ileocecal valve with resultant small bowel dilatation .Fig. 1PoInitial management consisted of non-operative measures as there was no evidence of hemodynamic instability, peritonitis or high risk of bowel perforation. Splenic arterial embolization (SAE) was completed with five 5 mm coils and six 2 mm coils followed by gelfoam alurry with approximately 90% splenic devascularization.nil per os. SheThe patient was subsequently admitted to the surgical intensive care unit for serial abdominal exams, abdominal x-rays and hematocrit level evaluations. She was made received a total two units packed red blood cells during admission. We administered mineral oil enemas in order to encourage fecal motility.CT of the abdomen and pelvis conducted three days post-SAE demonstrated diffuse hemoperitoneum, larger mesenteric-peritoneal hematoma but no significant change in the splenic hematoma—importantly, a small amount of colonic contrast appeared to migrate past the splenic hematoma.Oral diet was advanced as the patient’s bowel function returned to normal. Abdominal x-ray on post-SAE day six revealed contrast clearly migrating past the area of extrinsic compression consistent with report of normalized bowel function .Fig. 2AbThe patient was discharged home in stable condition after post-SAE vaccinations had been administered. At two-week follow-up, she required no further surgical intervention.3In 1994,Whittick and Viamonte described the first (and only) case of colonic obstruction caused by splenic hematoma . We presSimilar to the previous case, our patient is a sexagenarian who suffered a fall in proximity to the onset of abdominal pain and distension. Imaging revealed similar obstructive features; however, active extravasation of contrast was unique to our case and necessitated SAE. The SNOM hospital course was similar to the previously-reported management and appropriate in the setting of hemodynamic stability and absence of peritonitis.We opted to use mineral oil enemas to facilitate bowel movements under the assumption that solid stools proximal to the obstruction would be softened. Although this treatment appeared to produce an early bowel movement, the utility in the setting of extrinsic LBO remains unclear.The length of time necessary to allow return of colonic function is likewise unclear—the 1994 case resolved within a week, similar to the presented timeline, and we suggest grossly exceeding this time-frame represents failure of SNOM, presuming splenic hemostasis. We recommend consideration of invasive intervention  after this time period or if patient significantly clinically worsens.Close follow-up is necessary for any splenic hematoma including post-SAE as bleeding may recur or abscess may develop. Freitas et al describe several complications that may occur following SNOM of blunt splenic injury, including 3–16% gastrointestinal (GI) complications  ,7. Althoundergone SAE before developing GI symptoms. Additional cases would have to be analyzed to determine the actual risk of developing LBO during SNOM for splenic injury.Our patient remained hemodynamically-stable and without peritonitis throughout her hospital course and also in the outpatient setting. Overall, the natural history of LBO caused by splenic hematoma seems relatively benign.4We present the second case of LBO caused by extrinsic compression by traumatic splenic hematoma. Our case demonstrates the importance of considering splenic hematoma as an etiology of LBO, particularly in the setting of trauma. The management of this entity can be non- operative if splenic hemostasis has been achieved and in the absence of peritonitis and will likely resolve within one week.The authors  declare no conflicts of interests or disclosures.This work received no funding.Exemption from IRB approval was provided by the SBH Health System Institutional Review Board.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.GAB is the primary investigator and contributed to conceptualization, data curation, formal analysis, project administration, supervision and writing and editing of the manuscript.CK is the primary author and contributed to conceptualization, data curation and writing of the manuscript.CE is the secondary author and contributed to data curation and writing of the manuscript.VG, BC and TS contributed to data curation, validation, visualization and writing and editing of the manuscript.This does not seem necessary for Case Reports that are not first-in-man.Gerard A. Baltazar DO FACOS is the Guarantor of this work.This work complies with SCARE guidelines.Not commissioned, externally peer reviewed.


The present case report demonstrated the successfully radical operation (R0) for the highly advanced cholangiocarcinoma involving hilar hepatic arteries and portal vein, The careful preoperative diagnosis to define the adequate resection area and the expert operation was achieved without postoperative severe complications.A 55-year-old male was admitted to our hospital with obstructive jaundice, and the perihilar cholangiocarcinoma (PC) was found. At the time of finding PC, enhanced computed tomography showed the widely extension and involved the surrounding right hepatic artery (RHA) and bilateral portal veins (PV). According to extension of PC, left trisectionectomy combined resection of RHA and PV trunk was scheduled. By supporting plastic surgeon’s procedure, the scheduled R0 operation could be achieved and the patient was discharged without any severe complication but delayed intrahepatic abscess formation. After abscess drainage, he could immediately recovered and tumor relapse was not observed for a couple of months. By carefully preoperative examination, a complicated operation was successfully completed.The major hepatectomy with arterio-portal resections and anastomosis for advanced has been challenged at the high-volume center and the improvement of survival seemed to be obtained and, however, operative risk is still remained. This operation could be achieved by the expert surgeons under precise planning or management.The role of HBP surgeons is to challenge aggressive surgery even for patients with highly advanced local extension of PC. This case has been reported in line with the SCARE criteria [2A 55-year-old male was admitted to our hospital with obstructive jaundice. Imaging revealed a locally advanced PC, and endoscopic bile duct drainage and precise determination of the extent to which the tumor had invaded the intrahepatic bile ducts were performed. Contrast-enhanced abdominal computed tomography (CT) combined with three-dimensional imaging and cholangiography showed a perihilar mass lesion together with extensive stenosis of the intra- and extra-hepatic bile ducts a and b. No peritoneal dissemination, liver metastasis, or distant lymph node metastasis was seen after laparotomy. Although a hard and fixed PC lesion was present A, we attA microscopic examination revealed poorly differentiated adenocarcinoma with arterial and portal invasion, but the tumor did not extend to the dissected surface. No lymph node metastasis was found. It was histologically confirmed that an R0 resection had been achieved. The patient’s postoperative course was uneventful, and no severe liver damage was seen in the remnant liver. Therefore, the patient was discharged on day 24. However, after being discharged the patient developed an intra-hepatic abscess in segment 7, and perihepatic free fluid started to accumulate A and B, 3Recently, major hepatectomy combined with vascular resection has been performed for PC after adequate preoperative evaluations of the functional liver reserve . HoweverWith respect to the intraoperative findings and procedures, we first performed lymphadenectomy from the common hepatic artery lesion to the hepatic hilum, and then extended it along the relevant arteries to detect distant lymph node metastases. The dissected soft tissue around the dissection line was hard, but no cancer cells were observed, and furthermore, no lymph node metastases were detected during intraoperative pathological examinations. Eventually, a histological examination showed that there were no lymph node metastases. At present, combined resection and anastomosis of the hilar blood vessels tends to be performed after complete parenchymal transection of the liver in cases of advanced PC ,12. DuriFortunately, in the present case an R0 resection was achieved, and the patient’s postoperative course was good. However, delayed intra-hepatic abscess formation  occurred4We reported a case in which advanced PC was successfully treated using LT combined with arterioportal resection and anastomosis. An R0 resection and a good postoperative course were achieved; however, delayed intrahepatic abscess formation occurred. In such cases, precise determination of the extent of the tumor and evaluations of liver function should be performed preoperatively, and appropriate surgical simulations using three-dimensional imaging are important. Such efforts might contribute to expanding the surgical indications for hepatobiliary cancer and help to achieve safe R0 resection. Support by expert surgeons and physicians is necessary to achieve good patient outcomes in such cases.No COI.No funding.Ethical permission for case report is obtained at our intuitional policy.Informed consent was obtained in this patient. Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.All authors contributed the perioperative management and writing this paper. A.N., the first author, is a main operator and wrote this mainly.A.N., N.I., Y.T., M.H., K.Y., T.H, T.C., K.N.and Y.F. contributed patient operation and perioperative management equally in this case report. A.N. was chairman and director of the department and a main operator. H.I. supported the arterial anastomosis during operation. All authors approved the final version of the manuscript to be submitted.N/A.Professor K.N, who is a cardiovascular surgeon, who is another chairman of our institute.The authors declare that the work described has not been published previously, that it is not under consideration for publication elsewhere, that its publication has been approved by all authors and either tacitly or explicitly by the responsible authorities where the work was carried out, and that, if accepted, it will not be published elsewhere—including electronically in the same form in English or any other language—without the written consent of the copyright holder.Not commissioned externally peer reviewed.


Undifferentiated embryonal sarcoma of the liver is a malignancy with poor prognosis observed more frequently in children between 6 and 10 years old and very rarely found in adults. We present a case of embryonal sarcoma of the liver in a 60-year-old woman without significant medical history who presented to our attention with constitutional symptoms. Preoperative assessments did not show alterations in blood chemistry or tumor markers. Imaging studies showed a huge mass lying in the right abdominal quadrants, strictly adherent to the liver. The tumor was partially cystic with a thickened wall, sporadic contrast enhancement, and solid component. The patient underwent excision of the mass with associated liver bisegmentectomy S5-S6. Postoperative course was uneventful. The definitive histological diagnosis revealed the presence of embryonal sarcoma of the liver. We describe the clinical, histopathological, and therapeutic options adopted in the multimodal treatment of this disease. Undifferentiated embryonal sarcoma of the liver (UESL) is an extremely rare neoplasm in adults with less than 60 cases described so far in the literature –7. It isWe describe a case of a middle-aged woman with abdominal mass in the absence of specific symptomatology submitted to laparotomy with a final diagnosis of embryonal sarcoma of the liver.A 60-year-old woman came to our attention for abdominal pain, distension, and weight loss (about 6 kg in two months). Abdominal examination revealed a bulky mass occupying the right abdominal quadrants. She was submitted to laboratory routine tests without evidence of chronic liver disease and no alterations of serum tumor markers. Computed tomography (CT) scan confirmed the presence of an expansive mass (15 × 12 × 23 cm), poorly separable from the surrounding liver parenchyma, with heterogeneous contrast enhancement. The lesion was partially cystic with thickened walls and an intralesional solid component .After multidisciplinary discussion with radiologist and oncologist, indication to surgery was given and the patient underwent laparotomy. At exploration, a well-defined, partially cystic tumor was found originating from the fifth and sixth hepatic segments and adherent to—but not infiltrating—the right colon and omentum. En bloc resection of the mass and a S5-S6 liver bisegmentectomy were performed. The postoperative period was uneventful, and the patient was discharged on sixth postoperative day.The surgical specimen consisted of a lobulated yellow-reddish neoplasm sized 33 × 19 × 11 cm, with gelatinous cystic and hemorrhagic areas on cut surface. A fibrous discontinuous pseudocapsule separated the tumor from the adjacent compressed liver parenchyma. Microscopically, the tumor was composed of stellate or spindle shaped cells with bizarre morphology and ill-defined outlines, loosely arranged in an abundant myxoid matrix . ScatterAfter the definitive histological diagnosis and multidisciplinary evaluation, the oncologist proposed an adjuvant therapy of six cycles with vincristine, actinomycin D, and cyclophosphamide, which was accepted by the patient. 30 months after surgery, she is alive without signs of recurrence.Stocker and Ishak first introduced the term undifferentiated embryonal sarcoma in 1978 to describe a mesenchymal hepatic tumor without any sign of specific differentiation . A slighAlready in its first description, a mesenchymal origin was proposed and a possible precursor lesion was identified in mesenchymal hamartoma (MH) of the liver . SupportUESL may occur in any part of the liver but is most commonly found in the right lobe as a single, rapidly growing mass. Symptoms are directly correlated with the neoplasm's growth and include pain, abdominal swelling, weight loss, fever, anorexia, vomiting, diarrhea, lethargy, constipation, and respiratory distress , 10, 14.Radiological imaging is nonspecific but is generally able to detect a nodular mass in the liver. Ultrasonography usually shows a large mass with solid and cystic components which could be mistaken for an abscess or an echinococcal cyst. CT scan reveals an inhomogeneous mass with a hypodense component and eccentric abnormal contrast intake. Magnetic resonance imaging usually confirms the CT findings but is more useful for the identification of vascular invasion, biliary obstruction, and hilar adenopathy . PreciseThe macroscopic aspect of UESL is a nodular mass, which is usually solitary but may seldom be multiple and appears to be well demarcated with a fibrous pseudocapsule deriving from the compressed hepatic parenchyma around the lesion. On the cut surface, the neoplasm shows solid gray-white areas intermingled with gelatinous cystic spaces. Yellowish, poorly demarcated, necrotic areas as well as red-brown hemorrhagic areas are common. Microscopically, the neoplasm is composed of a population of round, spindle, or stellate cells with inconspicuous nucleoli and indistinct cellular borders in a myxoid matrix. Scattered cells with hyperchromatic and bizarre nuclei as well as multinucleated giant cells may be present. The mitotic index is typically high, and atypical mitoses are frequently observed. A peculiar morphological feature is represented by variable-sized eosinophilic globular inclusions in the cytoplasm and in the extracellular matrix. These are PAS-positive and diastase resistant , 15, 16.α1-antitripsine, and CD10, while negative for myogenin, CD34, CD117 (C-kit), hepatocyte paraffin 1 (Hep-par1), anaplastic lymphoma kinase 1 (ALK1), and S-100 [Immunohistochemistry is generally required in order to reach the definitive diagnosis and rule out other possible mimickers. A specific immunophenotype has still not be identified in UESL. Tumor cells usually express vimentin, desmin, CD68, B-cell lymphoma 2 (BCL2), nd S-100  and alsond S-100 , which ind S-100 .Differential diagnoses should be weighted considering the age of the patient, since each hepatic lesion presents more frequently in a certain age group. Lesions to be carefully excluded in children include hepatoblastoma, mesenchymal hamartoma, and embryonal rhabdomyosarcoma of the biliary tree. In the adult, the main differential diagnoses are with gastrointestinal stromal tumor (GIST), HCC with sarcomatoid aspects, and other high-grade sarcomas, especially leiomyosarcoma and angiosarcoma. GIST might be similar in the morphology of tumor cells, but classically express CD34 and C-kit in tumor cells . DistincIn the past, UESL had poor prognosis, with long-term survival rates lower than 37% , 21–23. Complete surgical resection with clear margins is currently considered the most important prognostic factor for UESL, both in children and in the adult. Patients with incomplete resection or rupture of tumor mass during surgical procedure have worse prognosis, with no statistically significant improvement in survival rates in those receiving subsequent chemotherapy . On the The major causes of nonresectability of UESL are multifocality inside the liver, portal vein involvement, and distant metastases at the time of diagnosis . NeoadjuIn summary, UESL in the adult population is very uncommon. From the clinical point of view, these lesions are mainly asymptomatic, they have no specific radiological features, and in most cases, preoperative diagnosis is impossible. Histological diagnosis is important in differentiating this group of rare mesenchymal neoplasms from the more common HCC and hepatoblastoma and may affect the choice of neoadjuvant chemotherapy for bulky unresectable lesions, suggesting the need of needle biopsy in such cases. The ability to achieve radical surgery with clear margins is a fundamental factor that influences the decision to resect the tumor or to refer the patient for neoadjuvant treatment. Liver resection with adjuvant chemotherapy should be offered to all patients with UESL, especially when there are positive margins or tumor rupture during the surgical procedure.


Lymphoepithelial pancreatic cysts are extremely rare benign pancreatic cystic lesions. High suspicion and an individual approach are imperative for the best management of those extremely rare entities. Lymphoepithelial pancreatic cysts are extremely rare benign pancreatic cystic lesions. High suspicion and an individual approach are imperative for the best management of those extremely rare entities. We report two cases of LECs, one patient that was treated with surgical resection and another patient that was managed conservatively. High suspicion and an individual approach are imperative for the best management of those extremely rare entities.. The clinical history and the histopathologic classification of the cystic lesions of the pancreas vary. They can represent benign, premalignant, or even invasive malignant lesions. Lymphoepithelial cysts (LECs) of the pancreas were first described by Luchtrath and Schriefers in 1985, and approximately 200 have been reported in the English literature so far. They represent true pancreatic cysts, and they are characterized histologically by mature squamous epithelium lining, surrounded by multiple lymphocytes or lymphoid follicles filled with keratinizing material.Cystic lesions of the pancreas are well‐known entities that have been recognized in up to 24% of the general population in various autopsy reports.2H. pylori‐positive gastritis. Thus, he was treated with proton pump inhibitors (PPIs) and antibiotic administration. Although his follow‐up endoscopy showed remission of the initial findings, the symptoms remained. He was afterward referred to our department. His physical examination revealed no particular findings apart from mild pain during the left upper abdominal quadrant palpation.The first case was of a 55‐year‐old, previously healthy, male patient. He presented with vague abdominal pain and discomfort after meals. He had a history of heavy alcohol consumption and smoking. His symptoms began 4 months before admission. Initially, he underwent an upper GI endoscopy that revealed an Patient's laboratory evaluation revealed elevated serum γ‐GT: 135 U/L, LDH: 250 U/L, and glucose: 165 mg/dL. Also, CA 19.9 was slightly elevated at 45 U/L. The rest of the laboratory values were within normal values. His upper abdominal ultrasound showed a mass located at the tail of the pancreas measuring 10 cm, and the computed tomography scan that followed revealed a cystic mass at the tail of the pancreas, with a max diameter of 12 cm  and magnetic resonance (MRI) imaging studies of the abdomen during the last decades has resulted in an exponential increase in the frequency of discovery of such lesions even in asymptomatic patients.Differential diagnosis of pancreatic cystic lesions is difficult, and physicians should be aware of the different types of cystic lesions and their characteristics before treatment selection. Pancreatic pseudocysts which are considered to be the most common nonneoplastic cysts of the pancreas do not represent true cystic lesions since their wall is not characterized by epithelial lining. Congenital cysts are rare and include those associated with genetic diseases such as autosomal dominant polycystic disease, cystic fibrosis, and von Hippel‐Lindau (VHL) disease. 90% of cystic neoplasms of the pancreas are represented by three types of lesions: Serous cystic neoplasms (SCNs) of the pancreas which due to the rare progression to malignancy can be managed conservatively provided that there are a definite diagnosis and absence of symptoms.Lymphoepithelial cysts (LECs) are rare benign lesions of the pancreas lined with stratified squamous epithelium and surrounded by mature lymphocytes. LECs are a rare entity, and their history is better described and researched in the maxillofacial region. They are also characterized as branchial cysts since they are believed to arise from embryologic remnants of branchial cleft origin in the neck.Due to their rare nature, lymphoepithelial pancreatic cysts are poorly characterized lesions, and their pathophysiology is not extensively studied. Different theories suggest that the formation of the cysts arises from squamous metaplasia of the pancreatic ducts or derivation from epithelial remnants in lymph nodes or possible displacement of branchial cysts during the pancreatic embryogenesis.The patients are usually male (80%) with a mean age of 55 years on presentation. They can be asymptomatic and have the lesion discovered incidentally during imaging studies performed for other pathologies (43%) or can present with a variety of symptoms with vague abdominal pain (48%) being the most paramount, whereas back pain, impaired general condition, jaundice, and various gastrointestinal and respiratory complaints are rare.Laboratory examinations are usually equivocal showing transaminasemia, cholestasis, or amylasemia in few cases 8%). There is a trend toward a rise of serum CA 19‐9 in half of the cases (50%) and a rise in CEA in 6% of the cases, whereas the rest of serum tumor markers remain in normal range.%. There On imaging studies, LECs are often round with a well‐defined wall. The wall is sharply demarcated from the pancreas and surrounding adipose tissue. The main radiographic finding that may help distinguish LECs from other pancreatic cystic lesions is that they are sharply demarcated from the pancreatic tissue. The cyst content may display a “caseous” appearance characteristic of keratinaceous debris or may be clear and serous.Cytological diagnosis of LEC through the use of EUS‐FNA is possible. EUS may reveal the cystic lesion on the pancreas raising the suspicion of a LEC diagnosis, while EUS‐FNA may set the diagnosis. On EUS examination, LECs appear solid, heterogeneous, and well‐circumscribed peripancreatic lesions. Cytological presence of squamous material and lymphocytes is diagnostic of LEC.Histological examination reveals a lined stratified squamous epithelium with an adjacent subepithelial layer of lymphoid tissue containing lymphoid follicles. Occasionally, mucinous cells have been present. Pathological analysis remains the gold standard for the diagnosis of pancreatic LECs.Surgery is still indicated in symptomatic patients or when malignancy cannot be excluded, as in our first patient. Pancreaticoduodenectomy and distal pancreatectomy are surgical options in large cysts locating in the head or tail of the pancreas. In smaller, well‐delineated lesions, cyst enucleation and drainage may be used if other macrocystic neoplasms have been excluded. Conservative management may be indicated in asymptomatic patients where a potential of malignancy has been excluded, as in our second patient, where “watchful wait” strategy may effectively avoid an unnecessary surgical resection.In conclusion, LECs of the pancreas are extremely rare entities. Although benign in nature, the differential diagnosis from other cystic pancreatic lesions with malignant potential is extremely difficult. Physicians should be aware of their existence, and high suspicion is crucial to exclude other malignant cystic pancreatic lesions. Both surgical resection and conservative management are effective strategies in managing LECs, and every patient should be approached individually for the most appropriate management.The authors declare no conflict of interest.All authors contributed equally to the manuscript.Written informed consent has been obtained from the patients.


In rare cases, rheumatoid pleural nodules can rupture into the pleural cavity to cause pneumothorax or empyema. We report successful surgical treatment of a patient with an intractable secondary pneumothorax due to rupture of a subpleural rheumatoid nodule into the pleural cavity.A 75-year-old man with a medical history of rheumatoid arthritis, acute coronary syndrome, and diabetes was admitted to our hospital because of left chest pain and dyspnea. A chest X-ray and chest computed tomography (CT) scan showed a left pneumothorax and several small subpleural nodules with cavitation. Repeated pleurodesis via a chest tube failed to improve the pneumothorax, so we decided to perform thoracoscopic surgery. Air leakage was detected in the left upper lobe where the subpleural nodule was visible on chest CT. Resection of the lesion successfully resulted in resolution of the air leakage. The final pathological diagnosis of the subpleural nodule was a pulmonary rheumatoid nodule. The patient has had no evidence of recurrence of pneumothorax after surgery.We obtained a final pathological diagnosis of a rheumatoid nodule that caused an intractable pneumothorax. Pneumothorax associated with rupture of rheumatoid nodules in the subpleural cavitary is difficult to treat with thoracoscopic surgery as a second-line treatment. Rheumatoid arthritis (RA) is the most common form of chronic inflammatory arthritis; RA can cause noncardiac thoracic complications, such as pleuritis, emphysema, pneumothorax, interstitial pneumonia, and rheumatoid nodules . NoncardA 75-year-old man was admitted to our hospital because of left chest pain and dyspnea. A chest X-ray showed a left pneumothorax Fig. , and a pPulmonary rheumatoid nodules occur in less than 1% of patients with RA . RheumatWe obtained a final pathological diagnosis of a rheumatoid nodule that caused an intractable pneumothorax. Pneumothorax associated with rupture of rheumatoid nodules in the subpleural cavitary is difficult to treat with thoracoscopic surgery as a second-line treatment.


Preputial calculus is a relative surgical rarity. It usually happens in elderly men with poor hygiene and uncircumcised penis complicated with phimosis. In the paediatric group, it is usually secondary to phimosis and other urologic and/or neurologic anomalies. Surgical treatment is the mainstay of treatment. Herein, we report a 27-year-old gentleman with preputial stone presented with obstructive uropathy and was successfully treated with surgical intervention. To the best of our knowledge, this is the first reported case of the largest preputial stone in Malaysia. Preputial calculus is a rare type of urolithiasis , 2. AlmoThe calculi are easily diagnosed via clinical examination as they are freely mobile within the preputial sac. They can exhibit as multiple as well as single stone as described in our reported case. Beside clinical assessment, a plain radiography is a useful modality to diagnose as well. The presence of radiopaque lesions proves their existences . Once thPatient with preputial calculi should be treated surgically to prevent complications. Herein, we report a 27-year-old gentleman with preputial stone presented with obstructive uropathy and was successfully treated with surgical intervention. He underwent circumcision and removal of the calculus. To the best of our knowledge, this is the first reported case of largest preputial stone in a young adult in Malaysia.A 27-year-old male with underlying congenital hydrocephalus and paraplegia was admitted to the hospital due to infected sacral sore. He had a placement of a ventriculoperitoneal shunt when he was a child. Upon admission, he also complained of progressively having difficulty in passing urine and leaked urination but he denied dysuria, hematuria, and pyuria. On examination of his genitalia, the prepuce was deformed and enlarged with phimosis. To our surprise, there was a huge stony hard foreign body under the prepuce measuring 5 × 5 cm in size . It was A pelvic radiograph was arranged and revealed a well-rounded radiopaque lesion in his penile region representing a large stone . Ultrasoth century. Patients typically present with acute urinary retention or obstructive uropathy [Preputial stone is an unusual entity of the urologic field. It is hardly found during our clinical practice nowadays especially beyond 20uropathy . Only a It is postulated that the formation of preputial stone happens after (1) inspissated smegma in uncircumcised penis, (2) stasis with the precipitation of urinary salts, (3) struvite composition secondary to infection, or (4) trapped stones during migration from the proximal urinary tract calculi. In this reported case, the most likely cause is the combination of the first two theories. Uncircumcised penis has higher risk of smegma accumulation in the preputial sac especially among those who are rarely practising a clean hygiene. The smegma itself acts as a local irritant which can cause inflammation. Unsolved inflammatory process will lead to chronic inflammation and ultimately cause scarring of the prepuce.Escherichia coli, Enterococcus sp., and Citrobacter sp. in urine culture of subpreputial space [This sac at the same time acts as a static reservoir for stone accumulation with metabolic action of urinary salt. It is common for stone to form in the prepuce especially among those with neurologic impairment and paraparesis or paraplegia as what has happened to our patient –6. Moreoal space , 2.Calculi are often palpable on examination of the prepuce; however, plain radiograph can confirm the existence. Ultrasound of KUB is essential to rule out any proximal stone as the treatment will include either shock wave, endoscopic or open surgery . Any sonThe mode of treatment involves removal of calculi and elimination of predisposing cause. As in this case, the patient underwent circumcision via dorsal slit procedure to remove the stone. Although preputial stone does not pose immediate life-threating event, it should be treated early and without delay . NeglectKnowledge on the importance of personal hygiene is utmost essential especially among uncircumcised individuals and neurological impairment. Encouragement on circumcision and emphasis on good hygiene should be disseminated to the public. Early detection and prompt action should be initiated early to prevent development of complications as mentioned above.


Glomus tumors are rare benign tumors which commonly affect the hand but are seldom seen extradigitally. Less commonly seen is the glomangioma, a variant of benign glomus tumor, and even rarer is the glomangiosarcoma, a malignant variant. Determining malignancy can be difficult and an intermediate diagnosis, glomus tumor of uncertain malignant potential, has been proposed. We present a case of a 56-year-old male with a recurrent forearm mass diagnosed as a glomangioma of uncertain malignant potential. Although the characteristics and behavior of malignant cases are still incompletely understood, it is important that a high index of suspicion be maintained when approaching these tumors, especially when large or recurrent. Glomangiomas should be included in the differential diagnosis when evaluating soft tissue masses in the forearm and should be evaluated for malignant features. Glomus tumors are rare benign tumors which commonly affect the hand but are seldom seen in other areas . They arWe present a case of a 56-year-old male with a recurrent forearm mass diagnosed as a glomangioma of uncertain malignant potential. Glomangiomas should be included in the differential diagnosis when evaluating soft tissue masses in the forearm, and one should be aware that glomangiomas can have malignant features.The authors have obtained the patient's informed written consent for print and electronic publication of the case report. The patient is a 56-year-old male who first noticed a mass on the dorsum of his left distal forearm in 1993. He recalls that the mass was generally painless but the “slightest external pressure” resulted in “instantaneous pain of great intensity.” He did not seek medical attention, and the mass gradually enlarged. In 1999, he was seen by an orthopedic surgeon who diagnosed a ganglion cyst and attempted aspiration. No fluid was obtained. Shortly thereafter, he was seen by a vascular surgeon who resected what preoperatively was felt to be a vascular hemangioma. However, the pathological examination was reported as glomangioma. After surgery, the mass was no longer visible but his symptoms did not abate. He continued to have exquisite pain with palpation over the surgical site. Over time, a mass recurred. He was first seen in our office in 2010, at which time, there was a 2.5 cm diameter mass at the dorsum of the left distal forearm. Surgery was scheduled and then cancelled by the patient who did not return until April 2015 at which time the mass had grown to 5 cm in diameter, still painful to palpation. He underwent excision of the mass in November 2015. Unlike after his first operation, the patient experienced complete resolution of his symptoms after excision of the mass. At 4-year follow-up, there is no evidence of recurrence of disease.A glomus body is a neuromyoarterial body found within the reticular dermis that functions as a specialized form of arteriovenous anastomosis and is responsible for thermoregulation. Wood first described a glomus tumor in 1812 as a painful subcutaneous nodule made worse by changes in temperature and cured by surgical removal ; the hisMultiple attempts have been made to categorize tumors comprised of glomus cells including solitary, multiple, solid, diffuse, adult, and pediatric. More recently, these tumors have been simplified into two major subtypes: the glomus tumor and the glomangioma ; howeverWhile many authors use the term glomangioma to describe multifocal lesions, in fact, the two entities show different clinical, etiological, and histopathologic features. The glomus variants are small, painful, and purple nodules with predilection for acral areas of the extremities, especially the nail beds of the fingers and toes . Aching Glomus tumors arise as sporadic tumors, and while glomangiomas may also be sporadic, autosomal-dominant inheritance patterns with incomplete penetrance, and variable expressions have been described –18. FamiHistopathologically, glomus tumors contain vascular channels surrounded by glomus cells. The glomus cells are monomorphic round or polygonal cells with large nuclei and scant eosinophilic cytoplasm. In contrast, glomangiomas contain more dilated venous channels than glomus tumors and resemble venous malformations. However, unlike venous malformations, they demonstrate single-to-multiple rows of surrounding cuboidal glomus cells , 19, 21.Glomangioma is the most common variant of glomus tumor and shows a more vascularized rather than solid growth pattern compared to glomus tumor. About 1% of glomus tumors and glomangiomas are reported to be malignant . In supeThe recurrence and metastatic potential of glomangiosarcoma has not been fully elucidated secondary to a paucity of cases in the literature. Variants of glomus tumor, including glomangiomyoma and glomangiomyopericytoma and symplastic glomus tumor, all are considered benign unless they exhibit the criteria for aggressive behavior listed above.Although glomus tumors and glomangiomas are a well-recognized cause of pain in the digits, they are often overlooked when formulating the differential diagnosis of extradigital lesions. Because extradigital tumors are more difficult to diagnose, patients often suffer from delayed diagnosis and/or misdiagnosis. According to several studies, the average duration of symptoms is reported to be between 7 and 11 years –26 and pWhile rare, glomus tumors and glomangiomas can be malignant. Although the characteristics and behavior of malignant cases are still incompletely understood, it is important that a high index of suspicion be maintained when approaching these tumors, especially when large or recurrent. Patients may present to a diverse group of physicians, including dermatologists, plastic surgeons, general surgeons, orthopedists, and pain specialists. It is important that all these health professionals maintain glomangioma in the differential diagnosis to ensure speedy diagnosis and treatment, as well as to be aware that there is a malignant variant.


This case seeks to highlight to endoscopists a rare benign disorder that may be encountered during endoscopy. Clinicians may be tempted to biopsy, which could lead to a catastrophic gastrointestinal haemorrhage.A 66-year-old asymptomatic Caucasian male was referred for colonoscopy with a positive faecal occult blood test as part of the UK national bowel cancer screening programme. Relevant past medical history included atrial fibrillation for which he took Dabigatran. He had a normal haemoglobin, mean cell volume, platelet and clotting function.During colonoscopy, an unusual vascular pattern encompassing the entire rectum extending to the rectosigmoid junction was noted at intubation. The lesion demonstrated confluent circumferential purple discolouration indicating venous blood supply, with heaping up of the mucosa involving the entire rectum and rectosigmoid junction. There was no corresponding history of venothromboembolic disease or liver disease. The patient proceeded to have computed tomography (CT) which revealed a considerably thickened rectosigmoid wall with multiple small rounded punctate calcifications within it, and no other visceral involvement. Subsequent magnetic resonance (MR) scan of the pelvis demonstrated extensive diffuse thickening of the rectum and lower sigmoid with intermediate to high T2 signal, and an internal architecture of multiple ‘grapelike’ lobulations.The findings were consistent with diffuse cavernous haemangiomatosis of the rectum (DCHR), an extremely rare benign submucosal vascular intestinal tumour originating from the dentate line. Misdiagnosis of DCHR is common and the macroscopic appearance of DCHR can mimic varices, haemorrhoids, polyps or proctitis. MR imaging is the gold standard for diagnosis. Common presentation is with haematochezia due to mucosal wall erosion. The treatment of choice for symptomatic DCHR is pull-through transection and colo-anal anastomosis. This case seeks to highlight a rare disorder that can be encountered incidentally during lower GI endoscopy. Injudicious biopsy is potentially catastrophic. In a patient who endoscopically has evidence of a DCHR, we advocate MR pelvis assessment to clarify the nature of the lesion to guide future management if required. The patient discussed remains well, asymptomatic, with no evidence of iron deficiency anaemia. This is a cautionary case for gastroenterologists who may encounter this rare benign vascular malformation during endoscopy and be tempted to biopsy, which could have severe consequences. The case provides an alternative differential to rectal varices, in the absence of portal hypertension.It also raises awareness of a condition that is associated with a significant lag between symptoms to diagnosis, despite diagnosis being straightforward with magnetic resonance imaging assessment.A 66-year-old Caucasian male was referred for colonoscopy having had a positive faecal occult blood test (FOBT) as part of the national bowel cancer screening programme performed in the UK. At screening the patient was asymptomatic and denied rectal bleeding. He was not anaemic.The endoscopist noted a past medical history of ischaemic heart disease, systemic lupus erythematosus, atrial fibrillation and hypercholesterolaemia, for which his medication included Dabigatran. The patient was a retired labourer, normally fit and well with a performance status of 1.2 insufflation and pump irrigation containing simethicone, under Entonox gas. At intubation an unusual vascular pattern encompassing the entire rectum up to the rectosigmoid junction was noted. At this point a decision was taken with the patient to proceed with full colonoscopy. The lesion was characterised by confluent circumferential purple discolouration indicating venous blood supply, with heaping up of the mucosa involving the entire rectum  to further evaluate this abnormality. The CT demonstrated a considerably thickened rectosigmoid wall with multiple small rounded punctate calcifications seen within it see Fig. . There wTo further characterise the CT findings a magnetic resonance (MR) scan of the pelvis was performed which demonstrated extensive diffuse thickening of the rectum and lower sigmoid with intermediate to high T2 signal, and an internal architecture of multiple ‘grapelike’ lobulations. The outer margin was well-defined, largely with a low signal wall indicating that the pathology was confined by the muscularis propria. Incidentally, the abnormality extended inferiorly, into the right side of the anal canal posteriorly, between the internal and external sphincter. Following enhancement, there was initially some nodular mucosal enhancement followed by progressive enhancement of the multiple discrete lobulations with infilling .DCHR is a rare benign vascular tumour of the large intestine, of unknown prevalence. First described in 1839, approximately 350 cases have been reported in literature to date . ColonicEighty percent of all colonic haemangiomas are cavernous, the other major type being capillary . HistoloDCHR appears to have an equal sex distribution, and symptomatic patients tend to present in youth . The vasMisdiagnosis of DCHR is common, with one Shanghai case series of 17 patients finding a mean delay time between onset of symptoms and diagnosis of 17.63 years . To compEvaluation of DCHR is best sought radiologically. Historically, barium enemas have played a role in identifying the length of the involved segment of colonic haemangioma. CT can depict the thickening of the rectosigmoid colon and pelvic phleboliths within the vascular sinuses of haemangiomas.MR imaging is widely regarded as the gold standard for radiological assessment of DCHR, in view of its superior soft tissue resolution and multi-planar facilities . The recMultiple therapies have been trialed with sclerotherapy, endoscopic mucosal resection and radiotherapy all failing to demonstrate long term therapeutic benefit. The treatment of choice for symptomatic DCHR is pull-through transection and colo-anal anastomosis, first proposed in 1976, and now successfully performed laparoscopically , 12, 13.This case seeks to highlight a rare disorder that can be encountered incidentally during lower GI endoscopy. Injudicious biopsy by the inexperienced practitioner, is potentially catastrophic. In addition, in view of his potential prostatic pathology, transrectal ultrasound guided biopsy of the pelvis was contraindicated. In a patient who endoscopically has evidence of a DCHR, we advocate MR pelvis assessment to clarify the nature of the lesion to guide future management if required, and to inform the patient  in the event of presentation to hospital with haematochezia. The patient discussed in this case reports remains well, asymptomatic, with no evidence of iron deficiency anaemia.DCHR is a rare but significant cause of rectal bleeding.DCHR should remain in an endoscopist’s differential as patients can wait many years to be diagnosed.If you suspect DCHR endoscopically, do not biopsy.MR imaging is the gold standard for radiological assessment of DCHR.


Extramammary Peget disease (EMPD) is a rare tumor, which typically occurs in the perianal regions. Perianal Paget disease (PPD) was first reported in 1893, and which has only 180 cases that have been reported in literature. The rarity of the disease means that no large studies have been made, and so the optimal treatment for this disease still remains controversial.In this case, we reported a 65-years-old female patient with PPD. The patient suffered intermittent pruritus in the perianal region for 1 year. She had neither genitourinary nor gastrointestinal symptoms. Local examination revealed a whitish gray skin lesion in the left perianal area with a 3 × 3 cm size.The perianal skin biopsy was consistent with EMPD. Then the patient underwent a screening colonoscopy, gynecological ultrasonography, and whole-body computed tomography to exclude underlying malignancy.The patient underwent wide local excision with margin control by frozen section examination and posterior thigh flap reconstruction. Subsequently follow-up remains 6 years.The operation was successful. The total operation time was 296 minutes, and the estimated blood loss was 120 mL. The patient recovered without any complication and discharged home on the sixth postoperative day. After 6 years’ follow-up, there was no evidence of recurrence and no influence in the anal bowel control function.PPD is a rare disorder; the current knowledge on diagnose and treatment is based on small case series. Thus, it is complicated to elaborate a consensus on diagnostic and treatment guidelines. Wide local excision remains the treatment of choice with a variety of adjuvant therapies. Our method has an advantage which is the posterior thigh flap could be designed in accordance with the defect of the perianal. It is mandatory that the patient must accept a long-term follow-up to detect local recurrence and to distant carcinoma. It has only 180 cases reported in literature, and so it is difficult to estimate the real incidence of PPDs.Extramammary Paget disease (EMPD) is a rare tumor that typically occurs in the perianal regions. The clinical presentation of PPDs is different from mild rash or roseola. Perianal itchiness and pain are the most common chief complaints, and other symptoms include bleeding, mucous seepage, lump, and difficulty in defecating.PPD is most often detected in old patients with similar morbidity in males and females.The diagnosis is made by pathohistology through the presence of specific tumor cells called Paget cells. The rarity of the disease means that no large studies have been done, and so the optimal treatment of this disease still exits controversial. It is generally recommended for the treatment of noninvasive disease that is wide local excision of the skin and subcutaneous tissue in the perianal region, and various techniques are described in the literature for the reconstruction of these defects.In the present report, we adopted the procedure that is wide local excision with margin control by frozen section examination. We performed a reconstruction technique to use a posterior thigh flap after resection of the perianal area.2A 65-year-old female came to our hospital in 2011 with history of intermittent pruritus in the perianal region for 1 year. The patient had neither genitourinary nor gastrointestinal symptoms, such as rectal bleeding, change in the bowel function, hematuria, dysuria, urinary frequency, or weight loss. Her family history was negative for skin, colorectal, or genitourinary cancer.Local examination revealed a whitish gray skin lesion in the left perianal area with a 3 × 3 cm size. The surrounding skin was lichenification Fig. . Rectal The perianal skin biopsy was consistent with EMPD. To exclude underlying malignancy, the patient was advised to undergo a screening colonoscopy, gynecological ultrasonography, and whole-body computed tomography (CT), which revealed unremarkable results. All laboratory examinations, including carcinoembrionic antigen, were normal.Informed consent was obtained from the patient for publication of this case report and accompanying images. After signing the informed consent, the patient was placed in a jack-knife position on the operation table after anesthesia. The operation consisted of a wide excision with frozen section control of the margins and a flap reconstruction. The perianal diseased skin and anal mucosa up to dentate line were integrally excised, preserving the external and internal sphincters. The size of the defect was 7 × 6 cm Fig. . For the3,6 Paget cells have the potential to invade and metastasize. The anatomic site of the PPD is strongly related to the underlying visceral carcinoma in 86% of cases. Our patients only have a skin lesion without associated colorectal carcinoma.PPD is an uncommon intraepithelial adenocarcinoma. The etiology of this disease is still not clear for now. Clinical feature is often nonspecific and insidious; thus, the diagnosis is frequently ignored. Patients commonly have symptoms like itching, irritation, rash, and pain, and bleeding may occur in long-term cases. These lesions typically appear in red or gray-white discoid dry bulge, and may turn into eczema or ulceration. The diagnosis could be determined through histological examination. The presence of Paget cells in the epidermis is direct evidence. Furthermore, CK7, CK19, and C-erb B2 are favorable immunohistochemical markers for the diagnosis of EMPD.2 laser ablation, imiquimod 5% cream and photodynamic therapy with topical aminolevulinic acid, but not enough data is currently available to define the role of noninvasive treatment. So, despite several controversies concerning PPD optimal therapeutic management, wide local excision of the skin and subcutaneous tissue in the perianal region is commonly adopted for the treatment of the disease. Spread of Paget cell could extend within the epidermis horizontally beyond clinical apparent disease.Treatment of PPD remains challenging and complicated by the nonspecific clinical situation and insidious onset of the disease. There is no standardized therapeutic guideline that we could choose. Myriad treatment options have been proposed for PPD, such as radiotherapy, chemoradiotherapy, CO As in our patient, on the edge of the defect, 6 points were taken for frozen-section analysis. However, wide local excision represents an aggressive operative management and usually results in a significant tissue defect which could not be covered up primarily without tension. Thus, it always causes remarkable morbidity and discomfort, and requires a special technique for its coverage. In most cases, primary suture or skin graft may not be suitable; the defect requires a flap transposition surgery. Moreover, reconstruction of perianal defects requires both the functional preservation and satisfactory cosmetic outcomes. According to the report, there are many techniques of flap transposition for covering the tissue defect areas, which have obtained satisfied outcomes, such as bilateral myocutaneous flaps of the gluteal or thigh muscles and V-Y island flaps and so on. The thigh offers great flexibility for the design of perforator flaps. Kishi et al reported the perianal reconstruction using a posterior thigh flap in 2010. They reported a satisfied outcome with this technique. In this case, we designed a posterior thigh perforator flap to cover the defect. The posterior thigh flap has shown advantages in the reconstruction of perianal defects compared to other flaps, such as the appropriate distance from the donor site to the defects, which makes the transposition easier. Furthermore, the well-supplied flap significantly increases the survival rate of the transplanted flap.Adequate microscopically clear margins are important to avoid clinical recurrence. when more than half of the perianal region have to be removed, or the radius of the excision is >3 cm. In this case, the patients were refused to do colostomy, so we inserted a 28-Fr rectal tube into the anal canal to diverse the fetal.Common complications include flap or graft failure, stricture and anal ectropion after local flap or skin graft. According to the extent of PPD, a colostomy may be required before wide local excision. Fecal diversion has been recommended by Shutze and Gleysteen, The margin of the old perianal lesion should be examined by punch biopsy every year, and the patient should be referred to colonoscopy examination every 2 years during the follow-up period.Long-term follow-up of PPD patients is very critical. The focus is on monitoring disease recurrence and development of an associated cancer.4PPD is a rare disorder. The current knowledge on diagnosis and treatment is based on small case series, which makes it complicated to elaborate a consensus on diagnosis and treatment guideline. Wide local excision remains the treatment of choice with a various adjuvant therapies. Our methods have the advantage that posterior thigh flap can be designed in accordance with the defect of the perianal. Long-term follow-up for detecting local recurrence and distant carcinoma is mandatory.K.S. participated in surgery, collected clinical information, and did the written work. H.L. and J.H. participated in surgery and collected the clinical data. Z.X. proposed and designed the procedure, and was the main operator in surgery.Conceptualization: Hai Luo.Methodology: Zhongshi Xie.Writing – original draft: Hai Luo, Jun Hu.Writing – review & editing: Kexin Shen.


Desmoid tumour (DT) is an unusual, benign tumour, more frequently observed in patients with familial polyposis and pregnant females. It usually presents as a single mass lesion, more frequently showing a compressive rather than an infiltrative growth pattern. We report a case of a 70-year-old male presenting with a multifocal, bilateral infiltrative DT of the perirenal tissue, with involvement of the choledochus wall. The patient was partly treated with tamoxifen and docetaxel, but both therapies were discontinued in accordance with the patient’s decision owing to mild toxicity; however, a CT examination performed 3 months later showed an unexpected remarkable reduction of the tumour at all sites. At 1 year follow-up, new pathologic tissue was visible surrounding the right renal pelvis and the calices. Desmoid tumour (DT) is an unusual, benign tumour, more frequently observed in patients with familial polyposis and pregnant females. It usually presents as a single mass lesion, more frequently showing a compressive rather than an infiltrative growth pattern. We report a case of a 70-year-old male presenting with a multifocal, bilateral infiltrative DT of the perirenal tissue, with involvement of the choledochus wall. The patient was partly treated with tamoxifen and docetaxel, but both therapies were discontinued in accordance with the patient’s decision owing to mild toxicity; however, a CT examination performed 3 months later showed an unexpected remarkable reduction of the tumour at all sites. At 1 year follow-up, new pathologic tissue was visible surrounding the right renal pelvis and the calices.–1); after an episode of thrombophlebitis, the patient asked to suspend tamoxifen and accepted a new therapeutic regimen (docetaxel 75 mg m−2 every 3 weeks); unfortunately, even this treatment was discontinued after only 4 weeks owing to neuropathy. A new CT examination was then performed to assess the results of the treatment. Unexpectedly, on venous phase images, the perirenal tissue showed a remarkable reduction on the left side and had almost disappeared on the right side , associated with specific mutations of the APC gene.5 The general incidence of DT in patients without a history of familial polyposis, such as in our patient, is 2–4 cases per million, with a sex ratio from 2 : 1 to 5 : 1. Sporadic DTs are more commonly observed in pregnant females (owing to a much-discussed role of oestrogens in pathogenesis) and in patients with previous major surgery.4 Our patient had previously undergone an exploratory laparotomy for gastric pain, cholecystectomy and hernioplasty 35, 30 and 15 years ago, respectively. DT usually does not metastasize and has a variable prognosis, alternating periods of progression and remission, and also shows a high tendency of recurrence after local excision. According to their localization, DTs can be classified as extra-abdominal  and abdominal. Abdominal tumours can have a superficial or intra-abdominal localization.4 The DT described in this report showed an unusual bilateral  as well as multifocal localization (peribiliary space); furthermore, the tumour had an infiltrative growth pattern, whereas most DTs compress contiguous structures, without real infiltration.3DT is a benign neoplasm, derived from a monoclonal proliferation of myofibroblasts.6 On CT scan, they can be either ill-defined or well circumscribed, with variable attenuation relative to muscle, and may or may not show enhancement after contrast media administration.7 MRI demonstrates low signal intensity relative to muscle on T1 weighted images, variable signal intensity on T2 weighted images and variable contrast enhancement. Low T2 signal intensity bands can represent foci of high concentrations of collagen deposition.6 In our case, the perirenal tissue was hypodense and ill-defined and did not show significant contrast enhancement; on the contrary, the tissue around the wall of the choledochus was markedly hyperdense after contrast injection.Imaging techniques are mainly used to define the tumour’s dimensions and the infiltration of contiguous structures to plan surgery. These tumours usually show considerable heterogeneity at imaging; as a matter of fact, different lesions, even in the same patient, rarely showed identical appearance, depending on the relative amounts of fibroblast proliferation, fibrosis, collagen content and vascularity. On ultrasound, DTs demonstrate low, medium or high echogenicity, with ill-defined borders.3 Clinical signs and symptoms depend on the tumoral site, dimensions and growth rate. DTs usually present as palpable or visible masses; pain and paraesthesia can be caused by compression of the nerves or muscles. In case of abdominal localization, severe complications, such as intestinal or ureteral obstruction, and fistulization, can occur, with worse prognosis.3Owing to this variable appearance, making a definitive diagnosis requires histological examination of the tissue samples. DTs are usually encapsulated unmoving lesions, similar to cicatricial tissues; an infiltrative growth pattern is less frequent. Microscopically, they consist of small, oblong cells with pale nucleus without atypias, separated by abundant collagen; macrophages, giant cells and lymphocytes are observed in peripheral areas. Moreover, DTs show expression of immunohistochemical markers typical of muscular tissues, such as actine, desmine and vimentin.8 Clinical and radiological monitoring with CT scan can be used for small and slowly-progressing tumours. Otherwise, surgery is the main treatment modality; the resection has to be wide, and the margin status seems to be the most important predictor of local recurrence.9 If the tumour infiltrates vital organs or structures, the main treatment modality is medical therapy consisting of administration of anti-inflammatories (such as celecoxib and sulindac) and the modulation of oestrogenic receptors owing to the recently admitted role of oestrogens in the pathogenesis.10 Approximately, 50% of DTs show complete or partial response to medical approach; in the presented case, we can presume that the tumour responded to tamoxifen, as such a good response would not have been likely after 4 weeks of docetaxel. Medical therapy can be used as an adjuvant to surgery; several trials have demonstrated that a combined strategy could reduce local recurrence, especially in advanced intra-abdominal disease. Chemotherapy and radiotherapy are also used in selected cases of unresectable tumours or as an adjuvant to surgery; in this regard, several tyrosine kinase inhibitors (such as imatinib or sorafenib) and cytotoxic chemotherapy  have been associated with clinical benefit in patients affected by DTs; however, this topic is still being debated, especially regarding the best chemotherapy regimens to be used. As a matter of fact, in the literature, reported results are mainly based on small single-centre case series studies; furthermore, in all these studies, the authors could not really affirm that disease stability was related to a benefit from chemotherapy and not simply to the natural history of the tumour.8 In our case, the patient’s poor compliance considerably influenced the medical treatment, which was intermittently and incompletely performed; nevertheless, this unusual bilateral multifocal tumour surprisingly showed a very remarkable response to therapy.There is no appropriate treatment for any DT; most authors currently advocate for an individualized/multimodality approach.DTs are benign neoplasms caused by monoclonal proliferation of myofibroblasts, more frequently occurring in patients with familial polyposis or Gardner syndrome, pregnant females and patients with previous major surgery.DTs can be classified as extra-abdominal and abdominal. Abdominal tumours can have a superficial or intra-abdominal localization, usually presenting as a single mass lesion.DTs usually compress contiguous structures, whereas infiltrative pattern of growth is less commonly observed.DTs can show a variable appearance on imaging, depending on the relative amounts of fibroblast proliferation, fibrosis, collagen content and vascularity.Surgery is the main treatment modality for DTs, even though most authors currently advocate for an individualized/multimodality approach.DTs show a variable prognosis, alternating periods of progression and remission, with a high tendency of recurrence after local excision.Medical therapy is mainly based on the administration of anti-inflammatories and the modulation of oestrogenic receptors, owing to the recognized role of oestrogens in the tumour pathogenesis.


Inguinal herniorraphy is a possible cause of iatrogenic seminal tract obstruction. Diagnosing and correcting these vasal injuries can be challenging. Successful re-anastomosis is technically challenging, with relatively low success rates. An uncommon alternative for selected cases is the crossover transseptal vasovasostomy. We herein report a case of a 36-year-old male patient with vas deferens injury after herniorraphy and a contralateral hypotrophic testis. He was successfully treated through microsurgical crossover transseptal vasovasostomy, with spontaneous pregnancy achieved, and the technique is presented in details. Inguinal herniorraphy is a possible cause of iatrogenic seminal tract obstruction. Vas deferens injury, estimated to occur in 0.3% to 2.0% of herniorrhaphies, is not usually recognized at the time of hernia repair . If the Diagnosing and correcting these vasal injuries can be challenging. The obstruction is commonly associated with significant scarring at the inguinal region. Successful re-anastomosis is technically challenging, with relatively low success rates –7. An un6/ mL; motility, 0%; and Kruger morphology 0%. Follicle stimulating hormone (FSH = 3.0mUi / mL), luteinizing hormone (LH = 3.9mUI / mL) and testosterone levels (350.1ng / dL) were within the normal range.A 36-year-old male patient with secondary infertility was admitted to our clinic. He had a 30-year-old wife with regular menstrual cycle and without gynecologic pathology on examination. He underwent right inguinal hernia repair three years prior to this first evaluation, after their first son was born. Sperm parameters were as follows: semen volume, 2.3cc; sperm count 3.0 × 10On his physical examination, right testicular volume was normal (11.7cc) and left testis was hypotrophic and retractile (7.3cc). No varicoceles could be detected neither on physical nor on ultrasound examination. His medical history did not reveal significant factors. Even though the patient wasn't azoospermic, the severe spermatic oligoasthenoteratospermia was incompatible with the prior pregnancy history. As the couple had a prior son and the only relevant factor was the inguinal hernia repair, it was suspected that a right vas deferens obstruction had occurred, and the associated left testicular atrophy was responsible for the low semen parameters. Facing the diagnosis, they decided to undergo intracytoplasmic sperm injection (ICSI); but after five unsuccessful procedures, with two unexplained miscarriages, the couple returned to attempt a surgical treatment.Surgical exploration of the testes was performed through a scrotal incision. After dissection of the vas deferens, a 23-gauge angiocatheter was placed into the lumen and contrast was injected to assure patency and confirm the diagnosis of obstruction . The cat6/ mL; motility, 6%; and Kruger morphology 1%. After 3 months, the couple got pregnant and the baby was born healthy after an uneventful pregnancy.After one month of the procedure, a control sperm analysis was performed, and revealed: semen volume, 2.3cc; sperm count 17.0 × 10Inguinal hernia repair is one of the most common causes of iatrogenic vasal obstruction. The true incidence is unknown, but for a procedure performed over 20 million times annually worldwide, the risks become high , 7. The When this unfortunate condition is suspected, there are limited diagnostic tools. If the vas is obstructed due to previous hernia repair, the epididymis may be thickened or indurated. Deferentography is conclusive although invasive, and normally performed intraoperatively. A testis biopsy can also confirm spermatogenesis on the obstructed side. Attempt to perform a vasovasostomy in the herniorraphy scarred tissue is cumbersome, with relatively low success rates , 4, 5. DAdvances in assisted reproductive techniques and ICSI have brought a new treatment modality for men with obstructive azoospermia. However, it is associated to additional risks and costs. Female factors, noteworthy female age, have to be meticulously considered before choosing the best approach for a specific scenario. However, if there is a solitary functioning testis with irreparable ductal obstruction, crossover transseptal vasovasostomy can be an alternative to restore patency, and might be offered as an option to these couples interesting in achieving pregnancy.


Introduction Three-dimensional (3D) technology is increasingly applied for planning challenging surgical interventions. We report our experience using 3D printing and virtual reconstruction for surgical planning of complex tumor resections in children.Methods Data were obtained from preoperative magnetic resonance. imaging analysis and 3D virtual recreations were performed using specialized computer software. 3D real-scale geometry models, including tumor, adjacent organs, and relevant vascularization, were printed in colorimetric scale and different materials for optimal structures discrimination.Results Four complex cases were selected. The first case was a bilateral Wilms tumor. The volumetric reconstruction proved the presence of enough healthy renal tissue, allowing bilateral nephron-sparing surgery. In the second case, reconstruction contributed to the location of pulmonary metastases. The third case was an abdominal neuroblastoma stage L2. The 3D model was of high value for planning and as a reference during the intervention. The last case is a cervico-thoracic neuroblastoma with an anatomopathological diagnosis of ganglioneuroma, located at the cervico-mediastinal juncture, in close relationship with the cervical vessels.Conclusions 3D reconstruction and the full-scale printing models are a useful tool in cases of complex tumor resections as they contribute to a better understanding of the relationships between the tumor and adjacent organs, helping to anticipate certain surgical complications. They also provide additional information to conventional imaging tests, being able to influence therapeutic decisions and facilitate the understanding by the family, improving doctor–patient communication. Surgical resection is one of the cornerstones in the multidisciplinary treatment of pediatric solid tumors. Preoperative tumor evaluation and surgical strategy planning are commonly based on conventional imaging techniques: ultrasound, nuclear magnetic resonance imaging (MRI), and computed tomography (CT). These tests give us images in two dimensions. Virtual three-dimensional 3D) reconstruction using specialized software provides a better understanding of the spatial relationships between the tumor and adjacent organs and allows exploring in an interactive form the surgical anatomy preoperatively. They also allow a fast and simple representation of high-definition 3D images, using files digital image and communication in medicine (DICOM) from CT or MRI.D reconstThis helps to evaluate more accurately the conditions under which the operation will be performed and to anticipate possible intraoperative complications.3D printing models, also called additive manufacturing models, are the result of the transformation of 3D digital files into tangible models.This technology began in the 80s when Charles W. Hull designed the first stereolithography machine. Although there are different types of 3D printer, all are based on the fusion or deposition of materials layer by layer until the production of the desired structure.Recently, numerous studies have been published documenting the usefulness of 3D technology in medicine both from the educational point of view and for planning and surgical training.In this article, we present four cases of pediatric patients affected from solid tumor lesions in which 3D reconstruction and printing technology was used in surgical planning, as they were cases in which the surgery was technically complex and or the complete resection presented an important forecast implication.We conduct a case series study. Data about complex oncological cases in which the 3D reconstruction and printing technology was implemented were collected from January 2016 to January 2018.The data for the 3D reconstruction were obtained in the DICOM format from the preoperative tests performed on the patients, MRI in three cases and CT in one, without any additional imaging tests. MRI was performed by 1.5-T scanner obtaining coronal and axial sections. Fast Spin Echo T2-fat-suppressed sequences were selected. The slice thickness was 4 to 5 mm. CT images were acquired by high-resolution multiplanar scan of 0.5-mm slice thickness.Subsequently, the specialized team of engineers of Cella Medical Solutions  performed the processing of two-dimensional images using previously validated medical image treatment advanced algorithms. Initially, a noise reduction algorithm (anisotropic diffusion filter) was applied, followed by segmentation algorithms of the anatomical structures of interest and creation of the 3D image of each structure. These images were exported in sterolitographic file (stl). Finally, the post-processing of the virtual reconstruction was performed.After virtual reconstruction was completed, the models were printed by fused deposit modeling (FDM) and injection printing 3D technology  . DifferThe printed models were made on real scale, including both the tumor and adjacent organs considered of interest. Special importance was given to the reconstruction of the vascularization involved.Between January 2016 and January 2018, data about four cases of patients affected by complex solid tumors were collected, due to both, the location and the relationships of the tumor, as the therapeutic implications of the surgical resection.Case 1: A 7-month-old infant was diagnosed with bilateral Wilms tumor. Neoadjuvant chemotherapy treatment was initiated according to the protocol of the International Society of Pediatric Oncology (SIOP) with little response to chemotherapy. In the preoperative MRI, a large tumor dependent on the left kidney with limited renal parenchyma was observed. Right renal tumor presented smaller size with largely conserved parenchyma. Initially, left radical nephrectomy and right nephron-sparing surgery were planned. The volumetric reconstruction  and one in the upper lobe of the left lung.Case 3: An infant of 13 months was diagnosed at 7 months of right abdominal neuroblastoma. In MRI, an extensive mass was observed that encompassed the regional aorta, the exit of the celiac trunk, the mesenteric artery, and the renal arteries, exerting mass effect on the inferior vena cava and displacing both the liver and the right kidney. Percutaneous puncture was performed with an anatomopathological diagnosis of poorly differentiated neuroblastoma, classified as Group “D” (low) of the International Neuroblastoma Risk Group (INRG) classification . The abdominal mass was considered unresectable, and an expectant attitude was adopted initially. During follow-up, hypertension and increased compression of the inferior vena cava were found. These symptoms were considered life-threatening; therefore, chemotherapy was initiated according to the European Low and Intermediate Risk Neuroblastoma protocol. The non-response to chemotherapy and the persistence of symptoms led to proposing surgery.Case 4: A 5-year-old girl was suffering from left paravertebral neuroblastoma. She was diagnosed prenatally of pleural effusion, and admission to the neonatal intensive care unit (ICU) was needed at birth due to respiratory insufficiency and thoracocentesis with diagnosis of chylothorax. During admission, a large cervico-thoracic tumor compatible with neuroblastoma was detected. The tumor included the left common carotid artery and left internal jugular vein without infiltrating them. Percutaneous biopsy revealed the diagnosis of low-risk undifferentiated neuroblastoma (group D) of the INRG classification . The tumor was considered unresectable (L2), and given the symptomatology of the patient, chemotherapy treatment was initiated with significant reduction in tumor volume. After that, expectant attitude was decided upon, while the patient remained asymptomatic. In the last year, a progressive increase in tumor size was observed, so a new biopsy was performed with a diagnosis of ganglioneuroma, and surgical intervention was considered.3D technology has potential applications in medicine among which is the creation of anatomical models to plan surgeries. In this article, we report our experience in the implementation of a new high-fidelity tool for, among other specialties, pediatric oncological surgery. The 3D virtual reconstruction and prototypes, designed from both MR and CT images in the form of DICOM files, have been useful in all four cases of complex oncological resections, not only when the surgery was being planned, but have also proved to be a useful tool during the surgical intervention. 3D virtual recreation computer software allows a 360-grade view of the tumor and the relevant adjacent structures in a clear colorimetric scale, as well as modified the consistency of the structures and removes or reintroduces them as the user desired. These kinds of software calculate precisely the volume of the tumor and the normal parenchyma as well. Real-scale printed models are able to be consulted and manipulated, to corroborate the distorted and particular anatomy and vascularization of each case. In the first case , the volumetric reconstruction was decisive at the time of the planning of the intervention, proving the existence of enough remaining renal tissue, which made nephron-sparing surgery in both kidneys posibleRegarding case 3, in which the celiac trunk was involved and both the liver and the portal vein are displaced, the usefulness of visualization and manipulation at the pre-surgical time and during surgery was also demonstrated. This set of technologies has been widely described in in liver surgery where precise evaluations of the remaining volume of the liver and anatomical variation are needed for the preoperative planning of safe curative hepatectomy.In surgical planning, a better understanding of spatial relationships can be obtained by exploring the surgical site interactively in a 3D format; preoperatively, this helps to evaluate more accurately the conditions under which the operation must be performed as well as to predict and prevent possible complications.There are already numerous publications and surgical series in which virtual reconstruction and 3D printing prior to surgery are routinely used in multiple specialties .As an added value, 3D models the patient and his family's understanding of the intervention that will be performed, improving doctor–patient communication.Our experience has been highly positive, and we believe that the application of both virtual reconstruction technology and the printing of physical models in pediatric oncological cases where complex tumor resection are required is a useful tool for improving outcome and will take a relevant place in the near future for planning and surgical training in this type of complex surgeries.