The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence-based medicine (EBM) techniques and aimed at improvement of the outcomes. Clinical questions (CQs) were decided based on the important clinical issues. For document retrieval, key words for literature searches were set for each CQ and literature published from 1980 to the end of September 2014 was searched in Pubmed, Cochrane Library, and Japana Centra Revuo Medicina (JCRM). The strengths of evidence and recommendations acquired by systematic reviews were determined following the Medical Information Network Distribution System (MINDS) technique. A total of 33 CQs were used to compile recommendations and the subjects included efficacy of resection, sclerotherapy/embolization, drug therapy, laser therapy, radiotherapy, and other conservative treatment, differences in appropriate treatment due to the location of lesions and among symptoms, appropriate timing of treatment and tests, and pathological diagnosis deciding the diagnosis. Thus, the Japanese Clinical Practice Guidelines for Vascular Anomalies 2017 have been prepared as the evidence-based guidelines for the management of vascular anomalies. The etiology of vascular anomalies on the body surface and in soft tissue are mostly unclear and no fundamental treatment methods have been established. Many patients visit many medical institutions seeking an expert, being a disadvantage in treatment. Hemangiomas and vascular malformations are frequently termed ‘hemangioma’, but these are different diseases in the ISSVA classification proposed by the International Society for Study of Vascular Anomalies (ISSVA) , 2, and ‘Clinical Practice Guidelines for Vascular Anomalies 2013’ (1st edition) Hermans et al.  treated t test showed a significant difference in the time until disappearance of the tumor, which was 8.7 and 22.4 weeks  in the treated and control groups, respectively. Temporary sleepiness/malaise was observed in 6 patients, irritable before falling asleep in 2 patients, coldness of the limbs in 6 patients, anorexia in 2 patients, and gastrointestinal disorders  in 1 patient, but no adverse event was noted in 9 patients.The mean time until complete cure of ulcer was 8.7 weeks, and those in whom the administration was initiated later (> 3.5 months) tended to require a longer time until cure than those in whom the administration was initiated earlier (p = 0.025). Also, analysis using the 2.Topical and/or systemic administration of antibioticsVercellino et al. , who staKim et al.  topicallWananukul et al.  topicall2 and examined the effectiveness of treatment according to the time until cure. The time until cure was 32.63 ± 13.06 days in superficial lesions, 42.89 ± 19.89 days in mixed lesions, and 57.03 ± 16.12 days in extensive lesions, with a mean of 40.09 ± 19.41 days in all lesions combined, showing significant differences among the 3 groups (p < 0.05). They also reported that the time until cure was significantly longer in larger (> 10 cm2) than smaller ulcers (p < 0.05).3.DressingsPandey et al.  treated 4.Laser therapyKim et al.  treated ® , wavelength: 585 nm, spot size: 5–7 mm, irradiation power: 5–6.8 J/cm2, pulse width: 0.45 ms) and reported that the number of irradiations until cure was 1 in 26 patients (68%) and 2 in 8 patients (21%) and that the mean period from the first treatment until cure of ulcer was 2.84 ± 0.22 weeks. Lacour et al. Surgical resection is one of the major treatment options performed for LM. Although LM can be cured by total resection, the objective of treatment is not necessarily total resection, because the disease is not malignant, and surgical resection is often carried out for cosmetic, functional, and symptomatic improvements. Cosmetic problems are considered to be particularly serious if the lesion is located in superficial areas such as the body surface and soft tissue. However, surgical resection has been known to cause complications including hemorrhage, infection, deformation, and nerve paralysis.In evaluating whether resection is effective, the balance between its positive aspects and negative aspects, such as complications, is important. For soft tissue/superficial LM, in which cosmetic improvement is important, problems including in what situations resection can be performed, whether there are criteria for the selection of resection, and, as there are differences in the incidence of complications, cure rate, and recurrence rate depending on the circumstances, whether its indications should be evaluated under different conditions are unclear. Therefore, the CQ, “Is surgical resection effective for soft tissue/superficial LM effective?”, was formulated, and the current knowledge was summarized.<Literature search and screening>As a result of literature search, 105 papers in Japanese and 348 papers in English were subjected to primary screening. Of these papers, 5 in Japanese and 42 in English were subjected to secondary screening concerning this CQ. They did not include papers with a high level of evidence, such as a systematic review and RCT, and all of them were case series or case reports. As a result, in the evaluation of this CQ, the results and discussion in each case series were integrated.<Review of observational studies (case series)>The effectiveness of resection of LM was evaluated from the following 5 viewpoints: (1) Effectiveness regarding the prognosis , (2) resection rate of the lesions (resectability), (3) functional outcome after resection (function), (4) recurrence rate (recurrence), and (5) complications.Generally, the rate of successful surgical resection is high, and ≥ 90% resection is reported to be possible in 60% or more of the patients –288. ThiIndications for surgical resection vary among papers, and differences in the patient background must be considered in the evaluation of the effectiveness of resection. While there were many reports that surgical resection was performed in combination with sclerotherapy, and resection is considered to have been performed when more favorable results were expected from resection rather than sclerotherapy, criteria for their selection are unclear. Therefore, there is certainly the large bias of individual variation in the circumstances, and it was clearly impossible to conclude that resection is uniformly effective.<Summary>While the effectiveness of surgical resection for soft tissue/superficial LM was evaluated, there was no literature with a high level of evidence. One of the major reasons is the diversity of the lesion type (cystic or cavernous), area of involvement, and history of other treatments. Because of this diversity, the condition of patients is considered to show an extremely wide variation, and their generalization is impossible. However, if conditions, such as the type of the lesion (cystic or cavernous), site of origin, and relationship with other treatments are restricted, tendencies were observed in functional prognosis, recurrence rate, and contents and incidence of complications.While the resection rate of lesions by surgical treatment was suggested to be generally high, selection criteria for resection were unclear. Therefore, it is speculated that resection was performed for patients clinically judged to be treated more effectively by surgery. However, since there were some serious complications of surgical resection that persist as sequelae, their possibility should be evaluated carefully in performing surgical resection. The risk of resection has been suggested to vary with conditions of the lesion. The functional outcome is poor, and the recurrence rate and incidence of complications after resection are high, in those that occupy a wide area and those that are accompanied by symptoms such as airway obstruction.From these observations, we propose at present, “While surgical resection is often effective, it must be selected in consideration of cosmetic aspects, prognosis, functional prognosis, resectability, and possibility of recurrence and complications.”, despite limited scientific grounds. If complete resection of the lesion is possible, surgical resection may be performed as the first-line treatment, but the possibility of other treatments including sclerotherapy, in particular, should be evaluated according to the diverse conditions of individual patients, and surgical resection should be performed when other treatments are ineffective or when surgical resection is considered clearly superior.Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)It is impossible to recommend optimal timing of surgery, and judgments according to the condition of each case are necessary.[Process of preparation of recommendation]Soft tissue/superficial LM are not malignant lesions. Emergent treatment may be necessitated by life-threatening symptoms, such as airway obstruction, but the initiation of treatment immediately after the diagnosis is generally considered unnecessary. The natural course of the disease differs considerably among individuals, particularly, in infancy, and the lesions may show a tendency of spontaneous regression but may also cause various functional problems due to rapid enlargement. Moreover, there are cosmetic problems characteristic of this disease in addition to functional problems, and early therapeutic effects are necessary to make social life comfortable. For these reasons, the selection of optimal timing of treatment, surgery in particular, is a major issue.For the selection of the timing of surgical resection, conditions to obtain the best results as well as indications for resection must be evaluated, and sufficient consideration of the balance between merits and demerits depending on the timing of resection is necessary. Therefore, in this CQ, we attempted to summarize the presently available knowledge about “What is the optimal timing of surgery for soft tissue/superficial lesions?”.<Literature search and screening>As a result of literature search, 67 papers in Japanese and 231 papers in English were subjected to primary screening. Of these papers 5 in Japanese and 42 in English were subjected to secondary screening for this CQ. They included none with a high level of evidence, such as a systematic review and RCT, and all papers were case series or case reports. Therefore, the results and discussion in each case series were integrated in the evaluation of this CQ.<Review of observational studies (case series)>Defining “the optimal timing of surgery” mentioned in the CQ as “the timing of surgery at which good results can be obtained”, we aimed to evaluate the timing of surgery at which resection is effective, problems, such as complications are few, and, i.e., “the best results” can be obtained as a whole. Conditions must be evaluated on the basis of the timing in addition to the effectiveness of surgery, but objective judgments were considered difficult in this evaluation. However, as it was considered possible to obtain information about the age and time of surgery from the literature reviewed in the previous CQs concerning the effectiveness, papers that evaluated the age at surgery were searched.Despite a careful review of the literature by secondary screening, there was no paper that analyzed cases from the viewpoint of optimal timing of surgery. There was information concerning the age at surgery, but its appropriateness was not evaluated. Papers that mentioned the timing of surgery are shown below.Concerning the timing (age) of surgery, unless the size of the lesion is small or there are symptoms that require urgent treatment, such as respiratory disturbance, it is recommended to delay surgery until the age of 3 years by expecting spontaneous regression or for the ease of identification of surrounding structures during surgery, ease of control of bleeding, and less problems with postoperative management . There wHowever, there was no paper that positively recommended resection without considering the time after the diagnosis or grounds for such a recommendation.<Summary>As a result of literature search for evaluating the CQ, “What is the optimal timing of surgery for soft tissue/superficial LM”, there were papers that mentioned the timing of surgery, but none of them objectively evaluated its appropriateness. Therefore, no suggestion about the appropriate timing of surgery was obtained from the literature available at present, but there were a few papers suggesting that the decision to perform surgery should be made carefully.Similar to the previous CQ, soft tissue/superficial LM of which the background vary in individual cases, and it is difficult to uniformly evaluate the effectiveness of resection. In clinical practice, in addition to medical reasons, social reasons including school attendance are considered to largely influence the decision of the time of resection. The results of RCTs are necessary to obtain objective data, but it is practically very difficult to arrange an RCT fulfilling the above conditions.While this CQ is a very important issue for patients and families as well as clinicians, there has not been objective evaluation of the optimal timing of surgery in the past. Presently, as quick decisions to perform surgery should be avoided, this guideline proposes, “The optimal timing of surgery cannot be decided in general, and judgments according to the condition of each case are necessary.”Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)A wide range of drugs are used for sclerotherapy. Although comparison among drugs has not been made, and consensus regarding the methods or frequency of their administration has not been reached, improvements are observed after sclerotherapy in various symptomatic, functional, and cosmetic (esthetic) aspects. However, complications including functional impairment have also been reported.[Process of preparation of recommendation][Literature search and screening]Concerning this CQ, 35 papers in Japanese and 92 papers in English  were retrieved. After their primary screening, 6 in Japanese and 18 in English were subjected to secondary screening concerning this CQ. Although they included 3 RCTs, many of the other papers were case series or case reports. Therefore, in the evaluation of the draft recommendation concerning this CQ, the results and discussion in each RCT and case series were integrated. While the evidence is scarce, the papers judged to be useful for the preparation of the draft recommendation are presented as review data.[Review of case series]A.SizeB.SymptomsC.FunctionsD.CosmeticsTreatment responsesComplicationsAs a result of literature screening, it was found that the effectiveness of sclerotherapy for facial microcystic LM has been evaluated from the following viewpoints.The contents of the accounts concerning the effectiveness of sclerotherapy are summarized according to these viewpoints.However, there were few reports that exclusively analyzed facial (and microcystic) LM, and lesions of the neck and other regions as well as the face were evaluated or different types of LM, such as cystic and mixed types, were reported together. In addition, the definition of the cavernous type and standard procedure of sclerotherapy (method and number of administrations) varied among reports, and these differences in the background should be considered in the evaluation of the effectiveness of sclerotherapy.ResponsesSizeThe sclerosing agents used for the literature search ranged widely from OK-432, bleomycin, ethanol, doxycycline, and sodium tetradecyl sulfate (STS). However, as none of the papers reviewed for the preparation of these guidelines evaluated differences in the effectiveness for facial microcystic lesions among drugs or the method or number of administrations of each drug, these evaluation items were excluded in discussing this CQ.Many of the papers that referred to the regression rate of the lesion classified the responses into (1) excellent or complete (regression rate ≥ 90%), (2) good or substantial (regression rate ≥ 50% and < 90%), (3) fair or intermediate (regression rate ≥ 20% and < 50%), and (4) poor or none (regression rate < 20%).Although there was no paper that collected cases of facial lesions alone, Yang et al.  reportedSmith et al.  reportedB.SymptomsThere was no paper that compared sclerotherapy and resection for facial microcystic LM.C.FunctionsThere is no literature that evaluated this item based on objective data, and few reports referred to symptoms themselves. The information was limited to the report by Chaudry et al.  that symD.Cosmetic aspectsRavindranathan et al.  performe2.ComplicationsCosmetic improvements are difficult to evaluate objectively. Poonyathalang et al.  administAs complications in the facial region, there are a large number of reports , 307–312As complications caused by sclerosing agents, skin ulcer and necrosis and nerve damage due to ethanol leakage, hypotension during anhydrous ethanol injection, and epidermal detachment due to doxycycline have been reported , 315. Ho[Summary]In evaluating the CQ, “Is sclerotherapy effective for facial microcystic LM?”, analysis was performed from the viewpoints of responses to the treatment in terms of symptoms, functions, and cosmetic (esthetic) aspects and complications, but few papers with a high level of evidence were found. While the degree of regression of the lesions by sclerotherapy varied widely, the size-reducing effect of the therapy was consistently small unlike that in cystic lesions. Some papers referred to symptoms, functional outcome, and cosmetic improvement, but they were insufficient for general discussion of sclerotherapy for facial microcystic LM. As complications characteristic of sclerotherapy, serious impairment may be caused by leakage of the sclerosing agent , and this point needs attention. Based on the above observations, it is difficult at present to evaluate indications for sclerotherapy against microcystic LM by formulating criteria. Therefore, for the future, it is considered necessary to evaluate the usefulness of sclerotherapy addressed by this CQ by designs such as RCT.Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)Although there are many reports that sclerotherapy is useful, there is the risk of complications, and careful judgments about matters including the resectability of the lesion and selection of the sclerosing agent are necessary.[Process of preparation of recommendation]LM is the most frequent lymphatic vessel disorder of the abdomen. Intra-abdominal lesions are estimated to account for 10–20% of all LM, and the selection of treatment is difficult depending on the site of the lesion. While surgical resection is expected to be effective, less invasive treatments are considered necessary in view of stress to the patient and the possibility of severe complications such as lymphatic fluid leakage and bowel obstruction. Sclerotherapy, which is a major treatment for LM, is considered to be less invasive than surgery. Although positive therapeutic effects are expected, sclerotherapy is known to induce marked inflammation. Whether it can be performed safely without negative effects including complications and its long-term effects are major clinical concerns. In addition, what therapeutic effects are expected or what complications should be anticipated after sclerotherapy for the intra-abdominal lesion is also unclear. Therefore, the CQ, “Is sclerotherapy effective for intra-abdominal LM?”, was formulated, and knowledge available at present was compiled.<Literature search and screening>As a result of literature search, 19 papers in Japanese and 38 papers in English  were subjected to primary screening. Of these papers, 2 in Japanese and 9 in English were subjected to secondary screening concerning this CQ. They included no papers with a high level of evidence, such as systematic reviews and RCTs, and all were case series or case reports. Consequently, the results and discussion in each case series were integrated in the evaluation of this CQ.<Review of observational studies (case series)>The literature concerning the effectiveness of sclerotherapy for intra-abdominal LM was reviewed from the viewpoints of (1) therapeutic effects  and (2) complications.The drugs used for sclerotherapy ranged widely from OK-432 to bleomycin, ethanol, doxycycline, STS, acetic acid, steroid/tetracycline, and 50% glucose solution. According to our review, there was no paper that evaluated the differences in effectiveness of sclerotherapy in the abdomen according to the drug type or administration method or number of administrations of each drug.Therapeutic effectsRegression rate of the lesionB.SymptomsRegression of lesions of intra-abdominal LM by sclerotherapy was mentioned in 5 papers , 316–319There were 3 papers that referred to symptoms of patients treated by sclerotherapy for intra-abdominal LM , 317.According to Chaudry et al. , of the 2.ComplicationsOliveira et al.  reportedThree papers specifically mentioned complications of sclerotherapy for intra-abdominal LM. There was no report of deaths due to treatment-related complications. Oliveira et al.  treated Sclerotherapy was often performed before, after, or during surgical resection, and papers that reported the results of sclerotherapy alone were few. There was no paper that directly compared observation without treatment, sclerotherapy, and surgical resection. Few papers analyzed intra-abdominal lesions alone, and many papers included lesions in other areas or evaluated lesions in different intra-abdominal regions including the mesentery, retroperitoneum, and viscera collectively.Moreover, differences in properties of LM, such as cystic, cavernous, and mixed types, their definitions, criteria for the selection of sclerotherapy  varied among papers, and few papers evaluated these matters separately.Such differences in the patient background and contents of treatment must be considered in evaluating the effectiveness of sclerotherapy. In evaluating this CQ, particularly, differences in morphology of LM and sclerosing agents were excluded.<Summary>The CQ, “Is sclerotherapy effective for intra-abdominal LM?” was evaluated from the viewpoints of therapeutic effect, symptoms/functions, and complications, but no paper with a high level of evidence was found. While sufficient regression of the lesion and alleviation of symptoms were achieved by sclerotherapy in some patients, the response rate varied among reports, and information was insufficient for general discussion of sclerotherapy. Concerning treatment-related complications, there have been reports of bowel obstruction associated with sclerotherapy, and attention to this condition as well as intracystic hemorrhage is considered necessary. However, there was no report of chylorrhea, which was reportedly caused by surgery.Based on the above observations, it is presently difficult to determine indications for sclerotherapy in intra-abdominal LM by setting up criteria, but as there was no literature that strongly ruled out intra-abdominal LM as indications of sclerotherapy, these guidelines propose, “Although there are many reports that sclerotherapy is useful, there is the risk of complications, and careful judgments about matters including the resectability of the lesion and selection of the sclerosing agent are necessary.” For the future evaluation of this CQ, validation by a design with a high level of evidence, such as RCT, is considered necessary.Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)Since there is risk of treatment-related complications, it is proposed to consider therapeutic intervention when the lesion tends to enlarge or has become symptomatic.[Process of preparation of recommendation]Intra-abdominal LM occasionally presents with severe symptoms, such as abdominal pain, giant mass, and bowel obstruction, but may also be asymptomatic and detected incidentally. Lesions may gradually enlarge and cause serious symptoms due to infection and intraluminal hemorrhage.Under such circumstances, whether patients with nearly asymptomatic intra-abdominal LM should be aggressively treated, or when intervention should be optimally performed during their long follow-up period are major problems that pose clinical dilemma. Therefore, the CQ, “Are patients with scarcely symptomatic intra-abdominal LM candidates for treatment?”, was formulated, and knowledge available at present was summarized.<Literature search and screening>As a result of literature search, 206 papers in Japanese and 237 papers in English  were subjected to primary screening. Of these papers, 6 in Japanese and 9 in English were subjected to secondary screening concerning CQ 26. They included no papers with a high level of evidence, such as a systematic review or RCT, and many of them were case series or case reports. Since 7 papers among them described asymptomatic LM, their results and discussions were integrated to answer the CQ.<Review of observational studies (case series)>Seven papers , 320–324The literature was screened, and papers addressing issues concerning therapeutic intervention for scarcely symptomatic intra-abdominal LM including “What symptoms they may present with if they are left untreated?”, “By what studies and how often should they be examined?”, and “What other treatments are available and how serious are complications or risk of each treatment?” were reviewed.From the literature reviewed, symptoms of intra-abdominal LM  areReported complications in treated cases include recurrence that required re-treatment , bowel oWhile there have been reports , 322 thaIt should be noted that many asymptomatic cases may be left unreported and some asymptomatic lesions that had been detected were treated. There is no study with a high level of evidence indicating explicit criteria concerning the age, site or situation about whether intervention should be made for asymptomatic intra-abdominal LM.<Summary>The necessity of treatment of a patient with intra-abdominal LM with few symptoms should be determined after evaluating the balance between the risk of treatment and non-treatment considering its site and size as well as patient age. However, since research on indications for treatments has been insufficient so far and serious complications after treatment have been reported, deliberate evaluation for each patient is mandatory. When observation is selected, periodic imaging studies are recommended to optimize therapeutic intervention by detecting enlargement of the lesion. Also if any symptom has developed during follow-up, intervention should be considered. For these reasons, the recommendation, “Since there is risk of treatment-related complications, it is proposed to consider therapeutic intervention when the lesion tends to enlarge or has become symptomatic.” was adopted.Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)Conservative treatments, such as fasting, high-calorie infusion, and medium chain triglyceride (MCT), should be performed first, but, if they are ineffective, drug treatment, sclerotherapy, and surgery may also be considered.[Process of preparation of recommendation]Refractory chylous ascites causes loss of large amounts of protein and lymphocytes, decreases in the blood lipid levels, and abdominal pain, unpleasantness, and dyspnea due to abdominal distention and markedly reduces the quality of life. The cause of ascites often remains unknown. Treatment of chylous ascites may require drainage to avoid abdominal distention. It is a very important point for clinicians to make proper judgments by understanding treatments and their effects and demerits. Therefore, it is considered beneficial to collect information about chylous ascites over a long period and compile guidelines. For this purpose, the presently available knowledge was collected by formulating the CQ, “What treatments are effective for refractory chylous ascites?”<Literature search and screening>As a result of search, 161 papers in Japanese and 728 papers in English  were subjected to primary screening. Of these papers, 15 in Japanese and 12 in English were subjected to secondary screening for CQ 27. They included none with a high level of evidence, such as systematic reviews and RCTs, and consisted of 1 multicenter and 2 single-center case series and case reports. Consequently, we used the results and discussion of 27 papers judged for the preparation of the draft recommendation were integrated although evidence was insufficient for the evaluation of this CQ.<Review of observational studies (case series)>As for causes of chylous ascites, congenital chylous ascites –342, idiWhen treatments are categorized, conservative treatments , drug treatments, sclerotherapy, and surgical treatment were performed.Conservative treatmentsThe results of review are presented below according to the treatment.Whether the amount of ascites changes by fasting should be checked first.High-calorie infusion is often used with fasting, and since there was no report that ascites increased under the effect of high-calorie infusion according to our review, it is recommended for nutritional support during fasting. In the multicenter case series reported by Bellini et al. , high-ca2.Drug treatmentsMedium chain triglyceride was used before, after, and during treatment , 348–352In drug therapy for chylous ascites, primarily octreotide  was used, and no report that discussed the effectiveness of other drug therapies was found by the present literature search.3.SclerotherapyIn the multicenter case series by Bellini et al. , octreot® in 1 Patients with intra-abdominal LM are treated with various modalities from non-surgical therapy to surgical procedures. Treatment modality is selected depending on the patient’s state. Therefore, it is necessary for the clinician, patient, and family to share information concerning complications that may be associated with treatments for implementing them smoothly. However, there are no resources that give a clear answer to this problem, and both clinicians and patients may become disconcerted. Therefore, the CQ “What kinds of complications are associated with treatments for intra-abdominal LM?” was formulated, and information available at present was accumulated and integrated for the answer.<Literature search and screening>As a result of literature search, 203 papers in Japanese and 602 papers in English  were subjected to primary screening. Of these papers, 23 in Japanese and 27 in English were subjected to secondary screening concerning this CQ. They included no papers with a high level of evidence, such as systematic reviews or RCTs, and all of them were case series or case reports. To answer CQ 28, the results and discussion in each case series were integrated.<Review of observational studies (case series)>Complications in the CQ were evaluated by defining them as those encountered when patients with intra-abdominal LM were treated, and reports on sclerotherapy and surgery were reviewed.Complications associated with sclerotherapy2.Complications associated with surgical proceduresSclerotherapy using OK-432 was reported to be associated with bowel obstruction and hemorrhage for mesenteric LM , and chyComplete resection of both mesenteric and retroperitoneal LM by laparotomy was reported to be associated with wound infection , 354 andIn a report  about coPartial resection by laparotomy was reported to be associated with persistent ascites over a long period that was refractory to the treatment .Patients with intra-abdominal LM are treated with various modalities including sclerotherapy and surgical procedures. Modalities were combined in many cases, and complications are often reported as those of entire treatment without more detail information about those associated with individual treatment.<Summary>For answering the CQ, “What kinds of complications are associated with treatments for intra-abdominal LM?”, no literature with a high level of evidence was found, but foreseeable complications were listed from many case reports. Bowel obstruction, hemorrhage, pain, hematuria, and chylous ascites were reported as complications of sclerotherapy. Serious conditions, such as occlusion of the inferior vena cava and massive resection of the intestine, as well as common complications, such as wound infection, bowel obstruction, hemorrhage and chylous ascites were reported as complications after surgical procedures.Although the incidences and differences in complications in respect of the site and histological type are not shown in the literature, each patient with intra-abdominal LM should be treated with sufficient evaluation of the site, size and symptoms. In addition, treatment must be implemented with sufficient understanding of the possible complications.Thus, we propose “Complications associated with sclerotherapy for intra-abdominal LM include bowel obstruction, hemorrhage, pain, hematuria, and chylous ascites. Surgical treatment of the disease can be associated with serious complications such as occlusion of the inferior vena cava and massive resection of the intestine as well as more common, wound infection, bowel obstruction, hemorrhage and chylous ascites.” as a recommendation draft.Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)Sclerotherapy is effective for macrocystic lesions, and surgical resection is effective for microcystic lesions. However, as the complication rate is relatively high, treatments should be selected according to the condition of each case.[Process of preparation of recommendation]Among LM, those that may cause airway obstruction due to their sites are life-threatening. Lesions in the mediastinum cause respiratory disorders if they physically compress the trachea or bronchi and obstruction the airway or markedly protrude into the thoracic cavity and narrow it.In such situations, aggressive and effective treatment is necessary, but the therapeutic approach must be selected carefully in consideration of the relationship of the lesion with the important organs around it such as the large cardiac vessels, mediastinal nerve, and thoracic duct. However, the judgment is often difficult in clinical settings.Therefore, the CQ, “What treatments are effective for LM causing airway obstruction in the mediastinum?” was formulated, and the presently available knowledge concerning matters including the risk of complications and prognosis of treatments, such as surgical resection and sclerotherapy, was summarized.<Literature search and screening>As a result of literature search, 134 papers in Japanese and 227 in English  were subjected to primary screening. Of these papers, 5 in Japanese and 16 in English were subjected to secondary screening concerning this CQ. Since they included none with a high level of evidence, such as a systematic review or RCT, and all were case series or case reports, the results and discussion in each case series were integrated.<Review of observational studies (case series)>By screening of the literature, the following approaches were found for the treatment of LM in the mediastinum.eppikajutsuto and ogikenchuto), and no treatment. Of these approaches, surgical resection and sclerotherapy using OK-432 have been evaluated in a relatively large number of cases, and reports of other therapy had extremely limited number of cases, e.g., reports of only 1 case.Therapeutic options are surgical resection, puncture and drainage, sclerotherapy , drug treatments EvidenceD (very weak)In a patient with LM around the airway, there is risk of respiratory distress in infancy, while airway obstruction is likely to be exacerbated by sclerotherapy. Particularly, when risk of airway obstruction is judged to be high or when the patient has already presented symptoms, it is proposed to perform sclerotherapy with sufficient preparations including airway management.[Process of preparing recommendation]LM of the neck, which are located in an exposed part of the body, may cause cosmetic problems that are important, but airway obstruction can particularly be a serious problem in some cases.Sclerotherapy, which is one of the major treatment modalities, is most effective in patients with cystic LM, but swelling of the treated portion after the therapy may cause or exacerbate airway obstruction symptoms especially in neonates. The upper airway will become less vulnerable to obstruction because it becomes less frail and wider as patients grow and respiratory distress tends to be unlikely. Therefore, it is occasionally difficult to determine how a patient who does not present any obstructive symptom should be treated in infancy.Thus, we evaluated this problem by formulating the CQ, “Should sclerotherapy be performed in infancy for a patient with the neck LM affecting airway?”<Literature search and screening>As a result of search, 86 papers in Japanese and 135 papers in English  were subjected to primary screening. Of these papers, 6 in Japanese and 20 in English were subjected to secondary screening concerning this CQ. They included 1 systematic review (SR), 1 RCT, 2 prospective studies (PS) and 1 retrospective cohort study, but all the others were case series or case reports. Therefore, the results and discussion, primarily, in these SR, RCT, PS, and retrospective cohort study, but also in other case series were integrated.<Review of observational studies>The literature concerning the effectiveness of sclerotherapy for head and neck LM in infancy was reviewed from the viewpoints of responses , size, symptoms, and cosmetic improvement) and complications.Sclerosing agents used as keywords for the present literature search varied widely and included OK-432, bleomycin, ethanol, doxycycline, STS and fibrin glue. No paper evaluated differences in effectiveness of various agents due to their methods of administrations for lesions around the neck affecting airway. Therefore, differences among agents were excluded from the evaluation of this CQ.ResponsesPrognosis B.SizeAccording to the SR by Adams et al. , the morMany of the papers that referred to the size regression evaluated it by four categories; (1) excellent or complete (≥ 90% regression), (2) good or substantial (≥ 50% and < 90% regression), (3) fair or intermediate (≥ 20% and < 50% regression), (4) and poor or none (≤ 20% regression).® in 2) and reported that the responses were good in 1 (20%) (cystic), partial in 1 (20%) (cavernous), and poor in 3 (60%) . However, they did not mention the evaluation criteria for good, partial, and poor.Ravindranathan et al.  treated According to the report of 8 cases with head and neck LM by Leung et al. , all patOgawa et al.  reportedCahill et al.  reportedC.SymptomsNehra et al.  reported®  in 2), 4 exhibited symptoms of airway obstruction before treatment. Symptoms included dysphagia in 2 and dyspnea (including croup-like symptoms) in 4 (some both). Symptoms were alleviated by sclerotherapy in 2 out of 4 , but tracheotomy was necessary in the remaining 2 (cavernous in both) without improvement.According to Ravindranathan et al.  who repoIn the report of 8 patients with head and neck LM and 5 patients with VM (aged 2 months–11 years) by Leung et al. , their sArimoto et al.  reportedKitagawa et al.  reportedD.Cosmetic improvementsNehra et al.  reportedNo paper has reported cosmetic results in detail. Only sporadically they mentioned about surgery for redundant skin after regression of cystic lesions by sclerotherapy.4. ComplicationsComplications associated with treatment for LM around the airway have been reported in many papers. They include temporary conditions caused by sclerotherapy such as fever , 365–371According to a systematic review about head and neck LM by Adams et al. , both neOgawa et al.  reportedKudo et al.  also repOn the other hand, Kudo et al.  reportedRegarding complications due to sclerosing agents, Cahill et al.  reportedThere are few papers that solely analyzed LM around the cervical airway. Most papers included lesions involving not only the neck but also the craniofacial and other parts of the body and reported LM with different properties such as cystic and mixed types. In addition, definition of cavernous lesions and methods of sclerotherapy  were not similar among papers, and differences in these backgrounds must be taken into consideration to evaluate the effectiveness of sclerotherapy.<Summary>The CQ, “Should sclerotherapy be performed in infancy for a patient with head and neck LM affecting the airway?”, was evaluated from the viewpoints of responses , decrease in size, symptoms, cosmetic improvements) and complications. Since there have been some reports on the risk of respiratory distress due to LM around the airway in infants, and therapeutic intervention is necessary even in infants when the risk is high or they have already developed symptoms. Such intervention is made by sclerotherapy or surgery, and as surgical resection is associated with the risk of more serious complications than sclerotherapy, intervention by less invasive sclerotherapy is recommended. Sclerotherapy is considered to be very effective because of high regression rate of the lesion and symptom/function-improving effect. However, its effect varies depending on the disease type, somewhat less effective in the cavernous and mixed types than in the cystic type. Furthermore, when it was performed for the lesions around the airway, it may be associated with the risk of exacerbation of airway obstruction symptoms due to reactive enlargement of the lesion. Thus, we formulated the recommendation, “In LM around the airway, there is the risk of respiratory disturbances from infancy, but airway obstruction is likely to be exacerbated by sclerotherapy. Particularly, when the risk of airway obstruction is judged to be high or when symptoms have appeared, it is proposed to perform sclerotherapy with sufficient preparations including securing the airway.”Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)Surgical resection is effective for reducing the size of the lesion and alleviating symptoms and functional impairment. However, total resection is often difficult, and careful decision is required in consideration of the possibility of complications and recurrence.[Process of preparation of recommendation]While the tongue is one of the frequent sites of LM, the lesion is often distributed widely over the neck rather than is localized in the tongue. LM of the tongue not only cause cosmetic problems, such as protrusion from the mouth and bleeding, but also readily occupy the oropharyngeal cavity and cause functional problems such as disorder of mouth closing, difficulty in speaking, respiratory disturbances, and impairment of oral food intake. These conditions are treated at departments including plastic surgery, oral surgery, otorhinolaryngology, and pediatric surgery. LM of the tongue are treated by surgical resection or sclerotherapy, but comprehensive evaluation of the condition of individual cases including the distribution of the lesion in the tongue, involvement of other areas and cyst components, and vascular distribution in addition to general information, such as the risk of complications and recurrence in each treatment, is necessary.Therefore, the CQ, “Is surgical resection effective for LM of the tongue?” was formulated, and the present knowledge about the effectiveness of surgical resection of the lesion, particularly, by partial glossectomy was summarized.<Literature search and screening>As a result of search, 29 papers in Japanese and 76 papers in English  were subjected to primary screening. Of these papers, 2 in Japanese and 10 in English were subjected to secondary screening concerning this CQ. They included 1 retrospective cohort study, but most other papers were case series or case reports. Consequently, in the evaluation of this CQ, the results and discussion of the cohort study and each case series were integrated.<Review of observational studies (case series)>The effectiveness of surgical resection of LM of the tongue was evaluated from the viewpoints of resectability of the lesion, symptoms, function, and cosmetic improvements as elements of responses as well as complications and recurrence.ResponsesResectability of the lesionTwenty-four cases of tongue lesions treated by surgical resection alone were reported in 4 papers. Catalfamo et al.  performeConcerning large lesions impossible to resect totally, Boardman et al.  reportedIn 1 case report, sclerotherapy was performed 15 times, but the lesion size could not be reduced, and surgical resection was performed, eventually resulting in a favorable outcome without recurrence .According to a report of 89 cases of head and neck LM by Lei et al. , the outB.SymptomsIn addition, a few papers –385 thatC.FunctionsA wide variety of symptoms have been reported depending on the site of the mass, and they include tongue discomfort, bleeding, pain, and difficulty in oral feeding . Roy et D.Cosmetic improvementsIn most patients who exhibited functional impairment, the lesions were so extended that they were no longer indications for one-time surgical resection. Large masses located at sites such as the tongue base cause respiratory disturbances, swallowing disorders, and difficulty in speech. According to the report by Azizkhan et al. , oral inObjective evaluation of cosmetic effects is also difficult.2.ComplicationsAzizkhan et al.  reported3.RecurrenceAlthough the properties of the lesions are unclear in some papers, facial nerve paralysis, vagus nerve paralysis, infection, hematoma, seroma, salivary leakage, ruptured suture, and skin flap necrosis have been reported as complications of the facial region. There have also been reports of temporary complications such as pain and hemorrhage.There have been a few postoperative evaluations reporting that no reactivation that clinically required treatment was observed. Lei et al.  reportedIn some papers, surgical resection was combined with other treatments –385, 387<Summary>Many papers suggest that surgical resection is effective for reducing the size of lingual LM. However, in patients with large lesions, lesions extending to structures other than the tongue, and microcystic lesions, several resections or combination of resection with other treatments such as sclerotherapy and laser therapy were necessary, and the recurrence rate tended to be higher. While a few papers referred to symptoms, functional outcome, and cosmetic improvements, none showed a high level of evidence, and the evidence was insufficient for general discussion of the effectiveness of surgical resection.Therefore, concerning the effectiveness of surgical resection for LM of the tongue, “Surgical resection is effective for reducing the size of the lesion and alleviating symptoms and functional impairment. However, total resection is often difficult depending on the distribution of the lesion, and careful decision is required in consideration of the possibility of complications and recurrence.” was proposed as a draft recommendation.Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)For chylous pleural effusion refractory to conservative treatments, surgical procedures, such as pleurodesis, ligation of the thoracic duct, and pleuroperitoneal shunting, may be effective.[Process of determining recommendation]Primary chylous pleural effusion during the neonatal period is often refractory and can be fatal. Thoracic drainage is performed for respiratory insufficiency due to accumulation of pleural effusion, followed by conservative treatments, such as nutritional therapy, steroid, and octreotide therapy, conducted primarily by neonatologist until resolution of chylous pleural effusion.In refractory cases that do not respond to these conservative therapies, surgical intervention, such as ligation of the thoracic duct and pleurodesis, may be performed. However, no sufficient consensus has been obtained concerning their effects. To evaluate problems, such as at what point surgical intervention should be made and whether aggressive surgical intervention is effective for such a condition, the CQ, “Is aggressive surgical intervention effective for chylous pleural effusion in the neonatal period?”, was formulated, and the knowledge available at present was summarized.<Literature search and screening>As a result of search, 98 papers in Japanese and 264 papers in English  were subjected to primary screening. Of these papers, 8 in Japanese and 9 in English were subjected to secondary screening concerning this CQ. They included none with a high level of evidence, such as a systematic review or RCT that evaluated surgical treatment, and all papers were case series or case reports. Consequently, the results and discussion in each of the case series judged to be useful for the preparation of the draft recommendation were integrated although they were weak as the evidence for the evaluation of this CQ.<Review of observational studies (case series)>The literature concerning the effectiveness of surgical treatment for chylous pleural effusion in the neonatal period was reviewed from the viewpoints of responses and complications.ResponsesSurgical treatment for neonatal chylothorax is performed in patients who respond insufficiently even to thoracic drainage in addition to nutritional therapy using MCT milk or total parenteral nutrition or drug treatment such as octreotide administration.The methods for surgical intervention found by the present literature review included ligation of the thoracic duct and pleuroperitoneal shunting as well as pleurodesis with OK-432 administration, intrathoracic infusion of fibrin, and povidone-iodine administration, and some patients diagnosed in utero underwent pleuro-amniotic shunting. Cases in which mildly invasive treatments, such as thoracoscopic ligation of the thoracic duct and intrathoracic fibrin application, have been reported in addition to those who underwent thoracic duct ligation by thoracotomy.Treatments that were performed before surgery and their periods were not uniform. In addition, since there are cases that developed chylous pleural effusion after surgery and those of congenital chylothorax, the diversity of the patient background must be taken into consideration in the efficacy evaluation.2.ComplicationsAmong the surgically treated cases, those in whom chylous plural effusion disappeared, respiratory symptoms were alleviated, and weaning from the respirator became possible have been reported , 389. InAs complications due to sclerosing agents, fever and increased inflammatory reaction due to the administration of OK-432 as well as pulmonary abscess and temporary flaccidity and protrusion of the upper abdominal region considered to have been due to intercostal nerve damage have been reported. While chyle leakage in the abdominal cavity was noted in a patient who underwent pleuroperitoneal shunting, there were no reports of fatal complications.Surgical treatment was performed in most reported cases when responses to conservative therapy were not obtained. Therefore, it must be assumed that the results of evaluation of this CQ are based on data concerning the effectiveness of surgery performed with conservative therapy.<Summary>The literature was reviewed concerning the effectiveness of aggressive surgical intervention for neonatal chylous thoracic effusion from the viewpoints of responses and complications, but no objective study with a high level of evidence was found. In most reported cases, surgical treatment was performed when responses to conservative treatments were poor. Therefore, it is difficult to compare surgery with other therapies, and the evaluation of the period of conservative treatment before surgery remains insufficient. However, there was a paper that proposed surgical intervention after attempting conservative treatments for 3 weeks as a standard.Thus, surgical intervention for neonatal chylous pleural effusion is characterized at present as an approach that may be effective but should be evaluated when the condition is not improved by other treatments, and “Surgical procedures, such as pleurodesis, ligation of the thoracic duct, and pleuroperitoneal shunting, may be effective for chylous pleural effusion refractory to conservative treatments.” is proposed as a draft recommendation.Recommendation:Strength of recommendation2 (weak)EvidenceD (very weak)While treatments including surgery, sclerotherapy, radiotherapy, nutritional therapy, and drug therapy are conducted, there is presently no effective treatment with a high level of evidence. Treatments should be selected in consideration of complications and adverse effects according to individual symptoms.[Process of preparation of recommendation]GLA and GSD are refractory diseases that cause a wide variety of symptoms in the entire body and are difficult to diagnose and treat. The investigation by the Health and Labour Sciences Research group (Ozeki group) carried out by 2013 showed that the mortality is particularly high when the patients had thoracic lesions.Among the thoracic symptoms, chylous pleural effusion/pericardial effusion are often refractory and occasionally fatal. While information about the disease is extremely limited because of its rareness, case reports are being globally accumulated as chronic cases are managed on an outpatient basis, and as severe cases are treated intensively.Presently, no radical treatment for these refractory diseases is known, but the CQ, “What treatments are effective for refractory chylous pleural, and pericardial effusion and respiratory disturbances of the patients with GLA and GSD?” was formulated to compile the knowledge about what treatments are effective as it is a problem of clinical importance.<Literature search and screening>As a result of search, 208 papers in Japanese and 617 papers in English  were subjected to primary screening. Of these papers, 2 in Japanese and 25 in English were subjected to secondary screening concerning CQ 37. They included no studies with a high level of evidence, such as a systematic review and RCT, and all were reports of 1-2 cases. Therefore, the evaluation of this CQ was performed by integrating the results and discussion in case series judged to be useful for the preparation of the draft recommendation despite the lack of evidence.<Review of observational studies (case series)>The effectiveness of various treatments for refractory GLA and GSD was evaluated according to the prognosis and the presence or absence of improvement in imaging findings, improvement in symptoms, improvement in airway obstruction, enlargement of the lesion, regression, treatment-related complications, recurrence, and reactivation.The cause of chylous pleural and pericardial effusion is lymphorrhea from lymphatic vessel tissue lesions that have primarily invaded the mediastinum and pleura, and lymphorrhea from osteolytic lesions of the ribs and vertebrae was also observed. Respiratory disturbances were caused by pleural effusion, chylous pleural effusion, pericardial effusion, and direct invasion of the mediastinum and lungs.As surgical treatments for chylous pleural effusion, procedures, such as thoracentesis, thoracic drainage, ligation of the thoracic duct, and pleural decortication, have been performed, and local lesions were surgically resected. In most cases, thoracentesis and thoracic drainage were performed, but chyle leakage was not resolved. As for complications, there were patients who developed hypovolemic shock and required blood transfusion and catecholamine administration or supplementation of albumin, immunoglobulin, and clotting factors –397. WhiAs a surgical treatment for pericardial effusion, pericardiocentesis was performed , 414–416As sclerotherapy, pleurodesis was performed using OK-432, talc, and minocycline , 416–418There have also been reports , 418–420Concerning nutritional therapy, fasting, high-calorie infusion, and MCT diet have been performed alone or in combinations, but few cases that showed alleviation of chylous pleural effusion were observed , 421.For drug therapy against chylous pleural effusion, drugs including interferon α, propranolol, anticancer agents , bisphosphonate, octreotide, steroid, sirolimus, and low-molecular-weight heparin were used. Interferon α was used most frequently , 421, anAlthough cases that responded to various therapies have been reported, treatments are often performed in combinations, and the evaluation of the effectiveness of each treatment alone is difficult at this point.<Summary>Treatments effective for GLA and GSD presenting with refractory chylous pleural effusion, pericardial effusion, and respiratory disturbances were evaluated by a review of the literature, which was primarily case reports. Various treatments, such as surgery, sclerotherapy, radiotherapy, nutritional therapy, and drug therapy, have been performed, but, there was no study with a sufficient number of cases and a high level of evidence because of the rareness of the disease and diversity of symptoms. Although cases that responded to various treatments have been reported, treatments are frequently performed in combinations, and the evaluation of the effectiveness of individual therapies is difficult at present. Sirolimus (a mTOR inhibitor) is considered promising as a drug for this disease, and some clinical trials are currently under way in Japan and abroad.In clinical situations, these diseases are not recognized as indications of various drug therapies by the Japanese health insurance system, and the therapeutic effects of other treatments are also uncertain. Therefore, the above treatments cannot be recommended, but we propose that treatments “should be selected in consideration of complications and adverse effects according to individual symptoms”. It is necessary to evaluate the invasiveness, complications, and adverse effects, and select the treatments judged to be appropriate for each case.The practice guidelines for vascular anomalies have been prepared as the evidence-based guidelines for the management of vascular anomalies.


A mediastinal mass in patients with a history of asbestos exposure should raise the suspicion of malignant mesothelioma. A mediastinal mass in patients with a history of asbestos exposure should raise the suspicion of malignant mesothelioma. A 73‐year‐old man was admitted to our hospital having progressive difficulty in swallowing solid foods and persistent cough. He was not a smoker and had no past medical history of cancer or infectious disease. However, the patient reported weight loss and a long‐term exposure to asbestos as a worker in a steel‐making industry. Oesophagogastroscopy showed a narrowing of the distal oesophagus Fig.  due to eAppropriate written informed consent was obtained for publication of this case report and accompanying images.


Lung cancer is the leading cause of cancer death in the United States, with more than 230,000 new cases, and approximately 150,000 deaths estimated for 2018. Lung cancer most commonly metastasizes to the brain, liver, lungs, bone, and adrenal system; however, there have been several cases of spread to soft tissues, with an incidence rate of approximately 0.75-9%. The objective of this case report is to highlight an unusual presentation of metastatic adenocarcinoma of the lung. In this case report, patient presented with a 3 × 3 cm soft tissue mass on the back. The mass was slowly growing but had become more painful and wished to have it excised. Preoperatively, the mass was suspected to be a sebaceous cyst but intraoperatively had deep attachments and other suspicious findings. Pathology had a positive immunoprofile for metastatic adenocarcinoma favoring a lung primary. Given this presentation of metastases, the prognosis is poor with a survival time decreasing to around 5 months. Overall, this case reinforces the importance of sending all soft tissue masses for final pathology with accurate labeling and the importance of immunohistochemical testing in aiding the identification of the primary. Lung cancer is the leading cause of cancer death in the United States, with more than 230,000 new cases, and approximately 150,000 deaths estimated for 2018 .Lung cancer preferentially metastasizes to the brain, liver, lungs, bone, and adrenal system . AlthougDiagnostic imaging of these masses is widely nonspecific as they most commonly resemble soft tissue sarcomas clinically, thus making biopsy necessary for definitive diagnosis . While tThis patient is an 81-year-old male with a past medical history of COPD, CKD, and atrial fibrillation and a history of pneumonia treated by his primary care physician and pulmonary medicine specialist 3 months prior to presentation. Patient also had a 46-pack year smoking history who quit in 1998. He presented to the office with a mass of questionable soft tissue origin in his back that was causing discomfort and pain. The lesion was reported by the patient as a firm, slow-growing, and bothersome for him to lean back in his chair or car seat. Lying on his back was also painful. Initial examination the mass was 3 × 3 cm and mildly tender to palpation with no accompanying lymphadenopathy, redness, or discharge. After discussion of the risks, benefits, and alternative options, the shared physician-patient relationship decided to undergo surgical excision of the back mass. Informed consent was obtained.The patient underwent LMAC anesthesia in the prone position with local application of 1% Xylocaine with epinephrine. An elliptical incision of the skin overlying the mass measuring was performed for grossly negative surgical margins. Circumferentially, the mass was taken down to the fascia and musculature. There were suspicious attachments of the mass to the surrounding musculature and fascia requiring excision for an en bloc resection. This was performed using cautery. The mass was removed, oriented, labelled, and sent to pathology.The tissue sample which measured 3.7 × 2.8 × 2.5 cm was stained at the margins with black ink and was noted to have a somewhat circumscribed fleshy appearing area measuring 2.3 × 2 × 1.5 cm. Microscopically, this appeared to extend to the stained ink margins. The sample was sectioned in blocks A1-A9 and subsequently immunostained. Block A5 displayed positive staining for TTF-1, CK7, and CDX-2, with negative CK20. This immunostaining leads to a diagnosis of metastatic adenocarcinoma with micropapillary features from a lung primary. The sample was noted to be an incomplete excision with transection of the mass at the peripheral margin.The patient was notified of the results, and oncology was consulted. Oncology recommends a staging workup and reexcision of the mass. The patient declined any surgical reexcision. The patient also refused to undergo further staging workup or medical or surgical treatment.As with all types of lung cancer, the patient's long smoking history put him at strong risk of developing the lung primary. However, preoperatively, the mass on the back was thought to be a benign sebaceous cyst. The patient denied any fever, chills, shortness of breath, hemoptysis, weight loss, or any concerning signs or symptoms for lung cancer. If the patient's history were concerning for lung cancer, a different preoperative approach would have been considered. This would include initially a chest radiograph. If concerning features are present, then proceed with a CT of the chest/abdomen/pelvis to assess the primary lesion and for adenopathy and metastasis. Blood work would also be obtained including CBC, BMP, and LFTs for liver metastasis, calcium, and alkaline phosphatase. If the patient reports headaches, a brain MRI can be considered. If the patient has elevated alkaline phosphatase or reports bone pain, a bone scan can be obtained for bone metastasis. Unfortunately, in this case report, the patient denied any symptoms concerning lung cancer, so no preoperative imaging or workup was obtained.This case presents the atypical manifestation of pulmonary adenocarcinoma metastasis as a soft tissue mass. Soft tissue metastasis is highly unusual in cancers of any origin with a prevalence of 1.8%. Of those resulting from a lung primary, adenocarcinoma is the histological type most commonly reported . TTF-1 iThere is evidence that pulmonary adenocarcinoma with micropapillary features, such as seen in this patient, may benefit from adjuvant chemotherapy after complete excision . DespiteAccurate radiographic and pathologic staging with both prognostic and therapeutic implications should be performed. In the past, MRI has been supported as a tool for diagnostic and treatment planning, despite its nonspecific nature. More recent studies have shown that F-18 FDG PET/CT scanning is more sensitive for both skin and soft tissue metastases . If our This is a case report of a soft-tissue mass of unknown origin causing pain and discomfort and noted on physical examination. Surgical intervention was chosen due to the location and superficial nature of the lesion. Upon excision, histological findings of the mass were indicative of metastatic pulmonary adenocarcinoma with micropapillary features with positive margins. With this presentation of metastatic pulmonary adenocarcinoma, the prognosis was poor and the patient declined to undergo further management. The case report highlights the importance of sending all soft tissue masses to pathology regardless of the preoperative diagnosis.


Traumatic bone cyst (TBC), a “pseudocyst” that usually affects long bones, is a rare lesion among cystic lesions in the jaws. The most commonly affected site is the posterior mandible. Most of the time, TBC is asymptomatic and discovered during routine radiographic examination. The treatment recommended for TBC is surgical exploration followed by curettage of the bony walls, which also serves as a diagnostic procedure. A 27-year-old Caucasian male with a noncontributory medical history was referred to our department for the endodontic evaluation of the mandibular right first and second molars, which were connected to an extensive asymptomatic osteolytic lesion. A multimodular diagnostic assessment involving CBCT imaging, ultrasound, and histopathologic examination led to a definite diagnosis of a TBC overlapping with apical periodontitis (AP). Subsequently, a multidisciplinary treatment approach was performed, including surgical excision and biopsy of the lesion, endodontic retreatment of the right mandibular first molar, and postsurgical root canal treatment of the second molar. During the follow-up period of five years, the patient was reassessed periodically once a year and showed, in the absence of signs and symptoms, progressive healing of the affected area. The present article reports a case following the CARE guidelines of a TBC combined with AP where a multimodular diagnostic assessment was performed and discusses the possible pathogenetic mechanisms involved in its generation. Although the majority of osteolytic lesions in the periradicular area of teeth are inflammatory in origin, some may not be inflammatory. The assessment of osteolytic lesions in the maxillary bones should always involve an exhaustive medical history report and a careful clinical examination comprising diagnostic tests and radiographic examinations that can be crucial in the differential diagnosis between apical periodontitis (AP) and nonendodontic lesions . Three-dA 27-year-old Caucasian male with a noncontributory medical history and no previous trauma was referred from the maxillofacial department of the hospital for the endodontic evaluation of the mandibular right first and second molars, which were connected to an extensive asymptomatic osteolytic lesion incidentally discovered on a routine panoramic radiograph and scheduled to be treated surgically under general anesthesia. The patient reported that he had experienced pain and swelling in the right mandible months before, while the first molar had a history of caries, extensive amalgam restoration, and incongruous endodontic treatment performed ten years earlier. The clinical examination showed no sign of buccal or lingual bone expansion, no lymph node involvement, and intact overlaying mucosa. The first mandibular molar was asymptomatic. The right second mandibular molar was asymptomatic with an intact crown, and it responded normally to the sensitivity tests at the time of our examination. The panoramic radiograph showed a well-defined unilocular osteolytic lesion with sclerotic margins located between the two molar teeth and superimposed on the alveolar inferior nerve with a slight displacement of the second molar. The first molar showed inadequate root canal treatment with signs of resorption in the apical third of the distal root involved in the osteolytic lesion. A preoperative periapical radiograph taken using the paralleling technique confirmed these findings . Cone beThe present report documents a case of a mandibular lesion diagnosed as TBC accompanied by complications, such as involvement in the field of a tooth affected by AP and an inflammatory granuloma-like histological pattern. TBC is described with a variety of names , suggesting a lack of complete understanding of its nature . Indeed,The clinical significance of this case report is the presence of two lesions, AP and TBC, combined in the same anatomical site, and such presentation has not been described before in the literature. The management of this pathological entity possibly was due to a multimodular assessment. In addition, this is the first report to describe the ultrasound examination of a combined lesion.


Cranial epidural hematoma is a serious event requiring immediate intervention. This can be due to sudden traction tearing the vessels between the dura and the skull. During posterior fossa surgery, brain collapse may emerge due to the sudden reduction of prolonged elevated intracranial pressure; it could cause dura-skull detachment to create epidural hematoma even far from the surgical site. Hence, we should be aware of this complication when approaching posterior fossa tumors as it frequently leads to severe neurologic impairment or death. Here, we report a 12-year old previously healthy child who was admitted with a 4-month history of severe headache, vomiting, and right eye blindness due to increased intracranial pressure. A brain Computed Tomography (CT) scan showed obstructive hydrocephalus, and contrast-enhanced Magnetic Resonance Imaging (MRI) confirmed intraventricular posterior fossa tumor. After tumor resection, the patient developed an epidural hematoma far from the surgery site. Removal of the hematoma exposed lacerations of superior sagittal sinus due to dural detachment. Failure to control intracranial pressure resulted in a fatal outcome. Cranial epidural hematoma (EDH) is blood collection in the space between the dura’s outer layer and the skull’s inner table . BleedinA 12-year-old previously healthy child presented with a 4-month history of severe headache, vomiting and blurred vision that progressed to right eye blindness. Fundoscopy reported papilledema and right optic nerve atrophy compatible with raised ICP. A brain CT scan showed obstructive hydrocephalus, and subsequent contrast-enhanced T1-weighted image revealed an intraventricular posterior fossa tumor with marked homogenous enhancing  was drained during the procedure. A midline sub-occipital craniotomy with the patient in sitting position revealed a well-defined pinkish lobulated mass inside the fourth ventricle. Gross total tumor resection was achieved without surgical complications or significant bleeding. The patient remained hemodynamically stable during surgery. Post-operative courseThe patient was managed in the Pediatric Intensive Care Unit under deep sedation due to high ICP. A control brain CT scan obtained 6 hours after surgery revealed complete removal of the posterior fossa tumor, collapsed ventricles (with right intraventricular catheter), and a large acute bifrontal epidural hematoma with interhemispheric bleeding far from the surgical site Figure . ExternaRemoval of the hematoma via bifrontal craniotomy exposed lacerations of the superior sagittal sinus due to dural detachment. A control brain CT scan obtained after 4 hours of the second surgery showed complete evacuation of the hematoma, important frontal pneumocephalus, more prominent ventricles (compared to previous imaging), and depressed brain cortex despite decompression Figure . The hisCranial EDH occurring remotely after tumor resection is rare . It mostThe proper mechanism for the formation of epidural hematomas is not well understood. EDH may occur after cranial surgeries, near the sites of craniotomies, or burr-hole sites. However, it is not common to see an epidural hematoma occurring distantly. These hematomas can occur at many locations (on the same side or the opposite side) and many of which were reported as occurring in patients with impaired CSF drainage due to hydrocephalus ,6.Most authors believed that EDH occurs when the vessels between the dura and the skull are torn because of the displacement or traction forces exerted on these areas. This traction occurs after a sudden drop in ICP with CSF decompression during posterior fossa or midline tumor surgeries. It can cause brain collapse progressing the hematoma with neurologic deterioration ending with coma and/or death if not managed properly and in time. Hence, the loss of cortical support and tears in blood vessels will result in subdural, epidural, or intraparenchymal hemorrhages. Tears in cortical veins during surgical procedures in sitting position, coagulopathies, hemodynamic fluctuations (rise in blood pressure during surgery), and position-related ischemia have also been reported as possible causes ,7,8.Cartier-Giroux et al. in 1980 reported a case of basal ganglionic hematoma following the resection of a cerebellar metastasis. They postulated that the hemodynamic disturbances that may occur due to irritation of the medullary vasomotor center during surgery might be responsible for transient hypertension [Surgeons preferred the sitting position as the approach used in our patient. It is hypothesized that the fluctuations in intracranial blood and CSF dynamics and the disruption of subcortical veins are important causes for EDH. In 1986, Seiler and Zurbrugg documented similar three cases and reviewed nine other cases performed in the sitting position . HoweverEDH post tumor resectionSupratentorial epidural hematoma after posterior fossa tumors resection are dreaded complications but are rarely reported  Table 11.The vessels between the dura and the skull are torn when the brain volume is massively reduced. The earliest information available about such a complication is that published by Tsugane et al. in 1976 when they suggested that a decrease in ventricular pressure by the ventricular tap during surgery may play an important role in the development of extradural hematoma . The corSeyithanoglu et al. suggested that in certain patients, the adhesions between the skull and dura are less important than dura-arachnoid adhesions and this would contribute to the development of a hematoma in the epidural area rather than the subdural space in cases of over-drainage . Hence, Ventriculoperitoneal shunts and ventriculostomiesEDH following ventriculostomies and ventriculoperitoneal shunt is a significant complication that could be due to excessive drainage, presenting mainly as subdural bleeding (incidence of 5-55%), where rapid decompression of hydrocephalus results in upward herniation of the infratentorial structures . FurtherThe most extensive case series of EDH (42 cases) following decompression of ventricles, was done by Odake and Matsumoto , which iPostsurgical pneumocephalus and high intracranial hypertensionPostsurgical pneumocephalus is often observed during the early postoperative period. It can be due to dural tear that allows air introduction via 2 possible ways: ball-valve mechanism from Valsalva  or vacuum phenomenon caused by CSF loss . TensionManagementA postoperative brain CT scan is routinely done, especially if the patient did not recover appropriately. Thus, postsurgical patient management is more efficient by maintaining a high index of suspicion and early detection of any complication . If a laRemote cranial epidural hematoma rarely occurs after surgery, but if it does, then it is a serious event that requires immediate intervention. Sudden traction on the vessels between the dura and the skull can cause them to be torn; however, the mechanism of formation is not entirely understood. It can be related to the rapid drop in ICP due to the reduction of CSF following excessive ventricular drainage or tumor excision.It is critical to remember such complications when suspecting hydrocephalus or performing surgeries in the posterior fossa with increased ICP. Even though such cases have rarely been reported in the literature, EDH carries complications and fatal consequences. Sometimes, patient management requires emergency surgery to relieve elevated ICP approaching the cause, as rapid bleeding evacuation carries beneficial outcomes, even for post-surgical silent or remote EDH. Consequently, a postoperative CT scan must be routinely done after surgical treatment, especially if the patient did not recover directly after surgery or experienced sudden neurological deterioration. Surgeons must recognize that surgical intervention alone might not be enough to control increased ICP; hence, neurointensive care is a cornerstone.Additionally, postsurgical pneumocephalus should not be diminished. We recommend the distinction of uncomplicated pneumocephalus from tension pneumocephalus as it is of vital importance as the latter constitutes a neurosurgical emergency that can result in fatal outcome as in our patient.


Echinococcus species, most commonly the Echinococcus granulosus. Although hydatid cyst can cause disease almost anywhere in the human body, it most commonly affects the liver and lungs. Primary musculoskeletal hydatid involvement is a very rare occurrence even in endemic regions. Musculoskeletal hydatid disease shows no pathognomonic clinical signs and symptoms. And the contribution of serology to the diagnosis is negligible due to the high rate of false-negative results. Therefore, radiological imaging studies have a critical role in the diagnosis of the disease. To the best of our knowledge, there are only a few case reports of primary hydatid involvement of the adductor magnus muscle in the current literature. Here we present a 55-year-old female patient with primary hydatid cyst in the adductor magnus muscle and discuss the case in terms of imaging.Human hydatid cyst is a zoonotic disease caused by the larvae of the  A 55-year-old female presented with painless swelling in the right thigh over the last few weeks. The patient had no history of fever, tremor, pruritus, or trauma to the thigh. On physical examination, there was a non-tender mass on the medial side of the right upper thigh the borders of which were not clearly distinguishable and did not show any signs of inflammation on the skin.T1 weighted images, the daughter cysts were hypointense and the mother cyst was iso-hyperintense as compared with the adjacent muscle tissue. Both the mother and daughter cysts showed high signal intensity on T weighted images. And T2 weighted images nicely delineated the hypointense ectocyst and hyperintense pericyst surrounding the lesion  . On T1 we lesion . The daue lesion . Followie lesion . ComprehAfter 5 days of anthelmintic treatment with albendazole, the cyst was surgically removed. No complications developed. In the pathological examination of the removed surgical material, internal germinal layers along with laminated membranes, which are diagnostic for HC, were found . A 6 monEchinococcus granulosus acquired through contact with carnivores, most commonly with dogs. Sheep are the most frequent intermediate hosts, and therefore Echinococcus granulosus is prevalent in the Mediterranean, Africa, South America, Middle East, Australia and New Zealand, the world's largest grazing areas.1 Although HC can cause disease almost anywhere in the human body, it most commonly affects the liver (65–75%) and lungs (25–30%). It rarely involves other sites such as the intra-abdominal structures, brain, heart, and musculoskeletal system (15%).2–4Human HC is a zoonotic disease caused by the larvae of the 4 Therefore, muscular HC is usually a secondary occurrence that develops as a result of migration of the larvae from the primary site after spontaneous or traumatic cyst rupture, rather than being a primary involvement. Accounting for less than 1% of all cases of hydatid disease, primary muscular hydatid disease is exceptionally rare even in endemic regions. According to the previous literature, the most frequently encountered muscles affected by the disease are the paravertebral, gluteal, and the lower extremity muscles.3–5 To the best of our knowledge, there are only a few case reports of primary hydatid involvement of the adductor magnus muscle in the current literature.6–9Contractility and high lactic acid content make the muscles unfavorable for HC.4 And the contribution of serology to the diagnosis is negligible due to the high rate of false-negative results. Therefore, radiological imaging studies have a critical role in the diagnosis of the disease.10 Ultrasonography, CT and MRI are imaging methods that can be used in both diagnosis and selection of the appropriate treatment approach. On ultrasonography imaging, a multivesicular cyst is diagnostic for the disease. However, HC can sometimes display different appearances such as a simple cyst, a cyst with floating membranes, or a complex cystic lesion. In case, there is abundant debris and/or inflammatory material inside the cyst, it may appear as a heterogeneous solid mass mimicking a tumor on ultrasonography examination.3–5 CT, another imaging tool, is not a preferred method for evaluating the cyst matrix, but it is accepted as the reference method for the examination of cysts showing calcification.10 MRI is a perfect method to evaluate the internal structure of the HC. In addition, it provides an excellent definition of surrounding musculoskeletal structures as well as the cyst borders, thanks to its multiplanar imaging capability.4,10Musculoskeletal hydatid disease shows no pathognomonic clinical signs and symptoms.11 Our case exhibited this characteristic rim which is made up of two layers: an inner hypointense layer representing the non-vascularized membrane belonging to the parasite (ectocyst) and an outer hyperintense layer representing the vascularized membrane formed in response to the host (pericyst)  . The vasher cyst .12 However, Oruç et al found no significant difference between ADC values of the simple cysts and those of Types 1 and 3 HCs. They reported that Type 4 HCs display significantly lower ADC values as compared with simple cysts and Types 1 and 3 HCs.13 The cyst we currently present showed a multivesicular configuration that fits the Type 3 HCs. And in contrast with the findings of Oruç et al, it exhibited a prominent restriction of diffusion in the mother cyst  values of the HCs are significantly lower than those of simple cysts, and they commented that diffusion-weighted imaging might help in the differential diagnosis of hydatid and simple cysts.her cyst . On the 4,11Treatment of muscle HDs is surgical removal with anthelmintic chemotherapy. Anthelmintic chemotherapy has been reported to reduce the rate of viable cysts within the lesion, thereby preventing relapse. It is important to remove the HC lesion as a whole without disintegration to prevent the infection from spreading to healthy tissue. It is best to remove the lesion using the same technique as the excision of a malignant tumor. An important issue to keep in mind when approaching any lesion with HC in the differential diagnosis list is that incisional biopsy and marginal excision are contraindicated in these cases. The liquid content of HCs contains a significant amount of toxic proteinous material, which is extremely risky for the host.Written informed consent was obtained from the patient for publication of this case report, including accompanying images.Although very rare, adductor magnus muscle can be the primary involvement site of HC. So, HC should be included in the differential diagnosis in cases where a well-defined soft tissue mass is detected in this localization.MRI is the method of choice in evaluating the internal matrix and extension of musculoskeletal HCs.T2 weighted magnetic resonance images is a useful finding for the differential diagnosis of musculoskeletal HCs.The rim sign on Before making definitive comments about the diffusion-weighted imaging features of musculoskeletal HCs, further case reports and comparative studies on the diffusion properties of the musculoskeletal system HCs are needed.


Isolated fallopian tube torsion associated with hydrosalpinx is a rare condition in the pediatric population. We present this unusual clinical case study in a sexually inactive girl.a12-year-old Caucasian girl presented symptoms of acute abdominal pain. Pelvic ultrasound revealed a normal looking uterus and ovaries and next to left ovary a imaging compatible with hydrosalpinx. She was discharged 48 hours later after clinical monitoring with oral analgesia and normal blood workup. At 3 weeks, she was readmitted for acute abdominal pain. Leukocytosis with left shift and raised C-reactive protein were observed. Her clinical condition worsened, and complication of the preexisting hydrosalpinx was suspected. Exploratory laparoscopy confirmed torsion of the fallopian tube. Left salpingectomy was performed. Histopathologic study confirmed a fallopian tube with hemorrhagic infarct.Torsion of the fallopian tube must be considered in the event of acute abdominal pain. Early diagnosis and trying conservative management with a view to preserving fertility in this group of patients are essential. Several intrinsic or extrinsic predisposing factors have been identified in adults as being associated with isolated fallopian tube torsion (IFTT) . HoweverHydrosalpinx can occur free of symptoms; however, when associated with IFTT, it is usually accompanied by symptoms of nonspecific abdominal pain . This coA 12-year-old Caucasian girl came to the emergency department of our hospital. She reported abdominal pain of 48-hour clinical course located in the left iliac fossa as well as feeling nauseous. The patient reported similar clinical symptoms, which were self-limiting, the month prior. She did not present with any medical or surgical history of note, and her gynecological history revealed that she had not undergone menarche and had not yet commenced sexual relations.3/μl) with left shift (81.2% neutrophils) and negative C-reactive protein . The results of basic analysis of normal urine and pregnancy tests were negative. We performed abdominal and transrectal ultrasound and visualized the patient’s uterus and ovaries of normal size, form, and echogenicity. Adjacent to the left ovary, an elongated formation of approximately 52 × 27 mm was seen, with anechoic content and negative color Doppler map, which suggested hydrosalpinx. No free fluid in the pouch of Douglas was observed , left shift (83.9% neutrophils), and elevated CRP (24 mg/L). Given these findings and suspected complications, exploratory laparoscopy was indicated. During the intervention, we observed that the left fallopian tube was increased in size, with torsion on its own axis, signs suggesting ischemia and impossible detorsion. We proceeded to perform left salpingectomy  may be useful according to personal history and ultrasound findings. Magnetic resonance imaging is a valid alternative but is less accessible and less efficient . HoweverIdeally, conservative surgery is attempted, opting for detorsion of the fallopian tube with a view to preserving the patient’s fertility, although this option can vary according to the surgeon’s choice . ClinicaIf, during exploratory laparoscopy, IFTT is confirmed, the surgeon should opt for conservative management or perform salpingectomy according to macroscopic characteristics, possibility of detorsion and signs of revascularization, in accordance with the algorithm proposed in a multicenter study . In our To conclude, IFTT has a nonspecific clinical presentation, and preoperative suspicions are rare. Pelvic abdominal pain must be considered as a differential diagnosis in children, especially if ultrasound study reveals a pelvic tubular structure without involvement of the ovary.Due to its rarity, case reports and small studies are the only source of information available in the medical literature. A long-term prospective study would be needed to determine whether future fertility is affected in cases of detorsion and revascularization of the fallopian tube.


Pseudodiphallia, as a rare kind of diphallia, which is characterized by a small accessory penile-like tissue without a normal penile anatomy structure. Only a few cases have been reported. Here, we report a case of pseudodiphallia with phimosis and bilateral extrarenal pelvis.A 23-year-old male visited our hospital with a complaint of external genitalia malformation. Physical examination revealed a normal-sized penis with phimosis, and an extra half glans horizontally attached to the right side of the normal glans penis is visible after completely retracting the foreskin. The CT urography showed a bilateral extrarenal pelvis, and no other abnormalities were found in the kidneys, ureter, bladder, and vertebral bodies.Based on the physical examination and the CT urography results, the 23-year-old male was diagnosed with Pseudodiphallia.Excessive penile tissue was resected, and a foreskin circumcision operation was performed under general anesthesia.The patient recovered smoothly without complications  after surgery. At 6 months follow-up, the patient was content with the external genitalia's appearance, and the urination and erectile function were normal.Pseudodiphallia is a rare kind of diphallia, and this is the first report on pseudodiphallia with a bilateral extrarenal pelvis. CT urography can be used to assess the associated internal anomalies before surgery. Pseudodiphallia, as a rare kind of diphallia, characterized by a small accessory penile-like tissue without a normal penile anatomy structure. Patients with pseudodiphallia usually do not present with ureteral or renal malformations or other congenital deformities.,5 Here, we report a case of pseudodiphallia with phimosis and bilateral extrarenal pelvis. Furthermore, we review previously published literature to further discuss the classification, etiology, and surgical methods of penile duplication.Diphallia (penile duplication) is a rare congenital malformation with an incidence of about 1 per 5 to 6 million newborns.2A 23-year-old male visited our hospital with a complaint of external genitalia malformation. Physical examination revealed a normal-sized penis with phimosis, an intact scrotum, 2 normally descended testicles, and a patent anus. Upon completely retracting the foreskin, an extra half glans, horizontally attached to the right side of the normal glans penis, became visible. There was only 1 urethral orifice, which was located in the middle of the normal glans penis. Both the normal penis and the extra half glans could be normally erected ,2.Hemiphalluses (each penis has corpora and a hemiglans),3.Pseudoduplication , and4.Partial duplication . Recently, it was described as the malformation of a normal penis with accessory penis-like tissue. This is the least serious category, treated by resection of the extra tissue with no need for urogenital reconstruction. The latest classification is more accurate to understand the essence and the operative techniques for diphallia.By comparison, the term “True penile duplication” corresponds to “True diphallia”, “Hemiphalluses” corresponds to “Complete bifid phallus”, and “Partial duplication” corresponds to “Partial bifid phallus”. The term “pseudodiphallia” was initially proposed by Villanova and Raventos, referring to “true partial diphallia”.3.2–16 Thus, the mortality of infants increases due to a higher risk of genitourinary tract infections. In the case of pseudoduplications and partial duplications, there are usually no associated severe malformations. Patients with pseudoduplication only have extra penile tissue attached to the normal penis. The patient we treated was examined and was found to have a bilateral extrarenal pelvis, which has not been reported in the literature before.True duplications and hemiphalluses, especially true duplications, are always associated with other congenital defects, such as bladder and urethra duplication, kidney anomalies, exstrophy of the cloaca and bladder, imperforate anus, colon and rectosigmoid duplication, vertebral and limb anomalies, and ventral hernia.3.3 MRI is a valuable method to accurately diagnose diphallia and associated malformations since T2-W images have a proper contrast resolution. In children, the erectile function can be tested by performing artificial erection with saline.A comprehensive and detailed examination should be performed before treatment. A physical examination can aid in the detection of surface deformities, such as exstrophy of the cloaca and bladder, vertebral and limb anomalies, and imperforate anus. Ultrasonography, voiding cystourethrography, magnetic resonance imaging (MRI), and CT urography are used to assess the associated internal anomalies, such as bladder and urethra duplication, kidney anomalies, and colon and rectosigmoid duplication.3.4 In the case of an actual penile duplication, partial duplication, and pseudoduplication, most surgeons choose to directly resect the hypoplastic duplicate penis, glans, or the accessory penile-like tissue in order to keep the main urethra. For hemiphalluses, more and more authors suggest joining the 2 penises without excision of excess tissue to preserve the excellent appearance and normal functionality of the external genitalia.Treatment should be based on careful consideration of aesthetics, function, and ethics. In cases with other malformations, individualized surgery is usually performed step-by-step based on the specific defects. In general, associated malformations should be treated first.3.5 It is generally accepted that duplication of the penis is caused by a lack of fusion of the paired mesodermal anlagen of the genital tubercle by the 15th week of gestation. Almost all karyotype analyses of patients diagnosed with diphallia are found to be normal. However, 1 report discussed an infant whose blood chromosomal analysis revealed a balanced chromosome 46, XY, t. The mothers blood chromosome analysis result was healthy, while his father was not available for a karyotype check. Although the first familial case of true diphallia was reported in 1994, diphallia is considered not familial or hereditary.The etiology of diphallia remains unclear; however, there are many possible embryological explanations. In normal physiological conditions, bilateral cloacal tubercles join each other at the anterior end of the pars phallic. Afterward, mesodermal columns grow around the lateral margin of the cloacal plate from the genital tubercle.Diphallia is a rare congenital malformation, varying from a small accessory penile-like tissue to complete penile duplication with other anomalies. The etiology of diphallia is unknown, and the treatment should be individualized based on careful considerations regarding the aesthetics, function, and ethics of the case. Pseudodiphallia is rare, but the least severe category of diphallia, which can be treated by resection of the extra tissue.Conceptualization: Nanze Yu.Data curation: Wenchao Zhang, Nanze Yu, Xiaojun Wang.Formal analysis: Nanze Yu.Investigation: Wenchao Zhang, Zhifei Liu.Methodology: Wenchao Zhang.Project administration: Nanze Yu, Xiaojun Wang.Software: Wenchao Zhang.Supervision: Zhifei Liu, Xiaojun Wang.Visualization: Zhifei Liu, Xiaojun Wang.Writing – original draft: Wenchao Zhang, Nanze Yu, Zhifei Liu, Xiaojun Wang.Writing – review & editing: Wenchao Zhang, Zhifei Liu.