A Morel-Lavallee lesion (MLL) is a rare internal denudement injury of skin and hypodermis from deep fascia, usually occurring hours to days after an inciting trauma. A common location is the pelvis or thigh where there is prominent vascularization and may mimic diagnoses such as deep vein thrombosis or contusion. Fluid collections that persist despite conservative management require surgical intervention and frequent and prolonged hospitalizations as in this case of a patient with a persistent MLL. We emphasize early imaging for diagnosis and surgical service involvement, as delay may lead to persistent symptoms and worse health outcomes. Morel-Lavallee lesions (MLLs) arise as a post-traumatic accumulation of hemolymphatic fluid between superficial and deep fascial planes . TangentThe trochanter and upper thigh are the most commonly involved regions where there is a rich vascular plexus supplying blood from the dermis to the fascia lata . LocatioMLLs are rarely seen in practice, as they often go undiagnosed or are diagnosed late . PrevaleWhile ultrasound is useful in identifying supra fascial fluid accumulation, computed tomography (CT) will definitively show the presence or absence of fluid . MeanwhiWe present a case of a 28-year-old male who presented with persistent pain and left leg swelling three weeks status post trauma. The inciting factor was a high-speed chase pursuit with a motor vehicle accident, and the patient was ejected through the front windshield as an unrestrained driver. Along with the rarity of MLLs, an interesting feature of the case includes a complex course despite appropriate imaging and standard-of-care treatment. He had recurrence requiring multiple incisions and drainage.A 28-year-old African American man with history significant for obesity, diabetes, and hypertension initially presented in October 2022 after being the pursuant unrestrained driver of a high-speed police chase, wrecking his vehicle and being ejected 50 feet from the car. He was admitted for contusions, strain injury, and traumatic rhabdomyolysis without fracture, and his primary conditions were poorly managed as an outpatient. His abbreviated injury scale as appreciated by trauma service was points for minor injury to head and neck (+1), minor injury to abdomen (+1), moderate injury to left lower extremity (+2), and minor external injury (+1). The injury severity score as calculated by the squares of each equaled 9 out of 75 total possible points, indicating overall minimal risk for complication and mortality. He had no personal or family history of coagulopathies and no labs indicating this effect. He had a normal coagulation panel and hemoglobin/hematocrit levels. Rhabdomyolysis improved with IV fluids and he was treated for cellulitis. At that time, there was clinical suspicion of a left medial meniscal tear due to an equivocal McMurray exam but no surgical intervention was performed due to the inability to confirm diagnosis.He presented three weeks later with left leg swelling and pain, ambulating with the use of a crutch and knee immobilizer, and had no infectious symptoms. He experienced persistent and worsening swelling in his left thigh with worsening pain over time. He denied pain in the calf and was able to ambulate at this time. Exam was significant for the hard left upper thigh which wrapped around medially to the knee and was associated with tense pressure. There were no external lesions or entry points for infection. Pulses were intact. Venous Doppler ultrasound was obtained to assess for compartment syndrome. Findings were negative for clot however concerning for extensive hematoma from hip to knee.CT extremity further defined the lesion as Morel-Lavallee Figure . SurgeryA week later, he presented with fluid re-accumulation, warmth to the area, fever at home, white count of 12.7 and lactic acid increase from 2.3 to 2.6, random blood sugar 298, and he was readmitted to the hospital for septicemia and drainage with possible wound vac placement. He had I&D of the left leg 50 cm long and 2 cm deep, from the knee to thigh, with similar findings of hematoma collection and was proceeded by wound vac placement (Figures He returned a week later with fever, nausea, vomiting, diarrhea, and abnormal drainage from his leg incision. Dehydration was evident on labs, he had acid/base derangements, and his comorbid conditions remained poorly controlled. Surgical incision was done, another wound vac, and partial complex closure, performed five times in the same hospitalization lasting a month complicated by cellulitis/wound infection related to methicillin-resistant Staphylococcus aureus infection. The infectious disease consultant recommended double-strength trimethoprim-sulfamethoxazole 160-800 mg oral tablet for 14 days of therapy at discharge. He remained hospitalized for the full month of December 2022, nearly three months after the initial injury and was discharged as stable again with HHC services. However, he has not been seen since in follow-up or records as of chart review October 2023.The MLL is an important trauma-related internal degloving injury that is important to follow from a surgical perspective due to its complexity when symptoms persist or worsen that may require surgical intervention. MLLs are often challenging to diagnose, as they can mimic other soft tissue injuries. The key to accurate diagnosis lies in a high index of suspicion, especially in the presence of a history of trauma in the case of our patient. Physical examination findings may include fluctuant, non-reducible swelling with occasional presence of overlying ecchymosis. However, these findings are non-specific and can be easily mistaken for other soft tissue injuries. Imaging modalities play a crucial role in confirming the diagnosis and assessing the extent of the lesion . UltrasoEarly recognition and prompt management of MLLs are essential to prevent complications such as infection, necrosis, and chronic seroma formation . TreatmeDespite the timely identification of the MLL through the utilization of ultrasound and CT imaging in our patient's case, the subsequent management proved to be challenging due to the persistent and complex nature of this particular MLL. Despite initiating multiple incision and drainage procedures followed by VAC placement, our patient experienced a complicated hospital course with ongoing challenges. Despite early intervention, the patient's condition ultimately necessitated multiple surgical debridements, washouts, and serial closure procedures due to chronic recurring seroma formation.Although MLLs are uncommon, it is imperative for physicians to maintain awareness of this disease entity. This case report's main objective is to make a meaningful contribution to the literature on MLLs, specifically by highlighting the importance of early intervention in an attempt to mitigate complications and improve patient outcomes. Additionally, we seek to provide insights into the complexities involved in managing MLLs despite intensive treatment, further emphasizing the critical role of timely intervention in optimizing clinical outcomes for affected patients.The patient in our case presented with clinical manifestations suggestive of an MLL, which was subsequently confirmed through ultrasound and CT imaging. Despite prompt intervention and recognition, the patient's hospital course was remarkably complicated, necessitating intricate surgical and procedural interventions. MLLs can pose a diagnostic challenge due to their nonspecific presentation, and their management can subsequently prove to be even more complex in nature. When left undiagnosed or untreated, these lesions are associated with considerable morbidity. Therefore, we emphasize the importance for clinicians to remain vigilant and knowledgeable about MLLs for early detection and timely management.


The lymphatic vascular system drains fluid, solutes and cells from the interstitial tissue surrounding the cells and subsequently returns the newly formed lymph back into blood circulation. Through their continuous drainage, lymphatic vessels maintain the adequate volume and composition of the tissue environment around the cells, guaranteeing their physiological behavior. Secondary lymphedema is a complex pathology whose progression depends upon an impairment of the lymphatic draining function, developing because of demolishing oncological surgeries, radiation, blood vascular malformations or surgical excision of local lymph nodes. In such cases, removal, obstruction, or impairment of the lymphatic vasculature cause the accumulation of fluid, solute and cell debris into the tissue, with progressive swelling, functional impairment and physical distress of the patient. The present review illustrates various cases of secondary lymphedema of increasing severity and the newest therapeutic solutions, spanning from indication of adequate body posture to physical therapy, to the newest and least invasive microsurgical approaches developed with long term satisfactorily results in the treatment of secondary lymphedema. Secondary lymphedema is a complex pathology which is very impairing to the patient, consisting of fluid accumulation in the tissue, accompanied by alteration of the interstitial fibrous tissue matrix, deposition of cellular debris and local inflammation. It develops mostly in limbs and/or external genitals because of demolishing oncological surgery with excision of local lymph nodes, or it may depend upon inflammatory or infective diseases, trauma, or congenital vascular malformation. Its treatment foresees various approaches, from simple postural attitude to physical therapy, to minimally invasive lymphatic microsurgery. This review focuses on the different types of evolving peripheral lymphedema and describes potential solutions to single objective symptoms. Particular attention is paid to the newest lymphatic microsurgical approaches, such as lymphatic grafting and lympho-venous shunt application, to successfully heal, in the long term, serious cases of secondary lymphedema of limbs or external genitals. The presented data also emphasize the potential role of minimally invasive microsurgery in enhancing the development of newly formed lymphatic meshes, focusing on the need for further accurate research in the development of microsurgical approaches to the lymphatic vascular system.  The vascular lymphatic system is specifically developed to guarantee the constancy of volume and solute composition of plasma and extracellular interstitial fluid. This target is achieved through a constant finely controlled removal of fluid, solutes of any size, and even cells from the interstitial tissue; the newly formed lymph is then carried along the lymphatic vessels to be eventually discharged into the venous blood. Lymph formation at tissue level requires that the hydraulic pressure be higher in the interstitium than in the lumen of initial lymphatics ,2; subse-Stage 1—subclinical lymphedema, due to anatomical or functional impairment of lymphatic network detected through lymphoscintigraphy, but with no clinical evidence of edema-Stage 2—characterized by permanent edema, which worsens with orthostatic posture and partly improves with clinostatic position and/or rest-Stage 3—permanent edema, not improving with clinostatic posture, and progressively deteriorating with lymphangiotic-erisipeloid and/or peri-lymphangiosclerotic events of the interstitial tissue-Stage 4—fibrolymphedema with columnar limb, pachydermia and cutaneous verrucoses-Stage 5—elephantiasis with limb deformation, functional impairment, and alteration of skin trophysm .Lymphedema is classified, based on its clinical manifestations and morphology, into five evolutionary stages: Depending upon the underlying cause, lymphedema can be further defined as primary or secondary . Primary(1)syndromic, when lymphedema is not the primary clinical evidence(2)with either pre-or postnatal systemic or visceral involvement(3)associated to growth, cutaneous or vascular abnormalities(4)congenital (5)with late onset .In addition, primary lymphedema phenotypes may be multifocal, depending upon edema localization, its clinical manifestations, and mutations of the genes involved in the expression of the disease . Based oAt variance with primary, secondary lymphedema does not depend upon genes’ mutations, but rather upon demolitive oncological surgery of bladder, uterus, breast, prostate or ovary, and of excision of pelvic, inguinal, or axillary lymph nodes. It may also accompany traumatic lesions and inflammatory or infectious diseases with involvement of major lymph nodal stations. Finally, impairment of otherwise healthy lymphatic collectors may be secondary to blood vessels’ malformation or pathways’ alterations. -in upper and lower limbs, the lymphatic network originates from initial lymphatics and, subsequently, merge into larger lymphatic collectors which carry the lymph to the axillary and inguinal lymph nodes, respectively. Through the thoracic duct, post-nodal lymph is eventually conveyed into the subclavian veins-lymphatics vessels draining the hypogastric area, the vaginal tunica, and the scrotal tissue discharge, respectively, into pre-aortic, para-aortic and superficial inguinal lymph nodes, respectively-lymphatic vessels originating from testicular tissue pass through the spermatic funiculus to reach the external iliac lymph nodes.Considering the localization and severity degree, lymphedema may heavily interfere with the patients’ social life, with episodes of self-imposed isolation, psychological denial of the medical significance of their condition, and rejection of proper specific treatment or follow-up. In this respect, it is worth noting that there are only few medical units specifically oriented to the multidisciplinary treatment of lymphedema. Indeed, although lymphedema is usually not life-threatening, its clinical approach may be very cumbersome, as most of the lymphatic vascular network is clinically inaccessible, due to its complexity, tiny dimensions, fragile structure, and depth into surrounding tissue. At present, only relatively large and superficial lymphatic-collecting vessels can be ichnographically identified and may therefore be approached, either with appropriate physical therapy or with extremely specialized surgery. In particular: The following sections describe various types of chronic localized lymphedema of increasing severity and focus on the physical therapies and the newest frontiers of lymphatic microsurgeries available for successful, long-term treatment of patients. At present, clinical treatment of primary lymphedema almost exclusively consists of physical therapy. In fact, its multifocal expression and our still limited knowledge of its pathogenesis precludes the guarantee of any other indication. However, rare patients with congenital lymphedema, if over 35 years old, negative for genetical aberrations, and with only minor lymphatic vessels defects , may benefit from surgical procedures. Excluding the other already mentioned etiologies of peripheral lymphedema, we would like to start describing an extremely rare example of local lymphedema, due to a blood vascular morphological malformation and vessels’ pathway alteration.Intrinsic artero-venous malformations and/or vessels pathway alterations due to embryonal angiogenetic mutations of distal vascular system usually do not lead to lymphatic insufficiencies, particularly when the vascular defect pertains to small vessels. However, lymphatic impairment may sometime develop and require clinical attention, such as in case of a transient secondary lymphedema of the neck, due to an extremely rare chronic retromandibular vein course, in a patient with known idiopathic double vena cava syndrome. The retromandibular vein is a major vein of the face, resulting from the confluence of the head and neck veins . It descends in the substance of the parotid gland and, subsequently, divides into: (a) an anterior branch, which travels forward and joins the anterior facial vein to form the common facial vein, which, at last, drains into the internal jugular vein; (b) a posterior branch, which joins to the posterior auricular vein to form the external jugular vein. In this case, a 39-year-old woman in uneventful status came to our attention with transient left latero-cervical swelling. The main genes involved in primary lymphedema  were excluded. A vague relapsing edematous symptomatology appeared for the first time when she was 30 years old and occasionally emerged: (a) in clinostatic, either right or left, lateral decubitus, with the ipsilateral limb stretched over the head; (b) in orthostatic posture, with prolonged rotation to the right of the head-neck, right limb abduction, or inclination of the head to the left. Tissue swelling of left latero-cervical and supra-clavicular area, associated to patient discomfort, began after about one hour and spontaneously disappeared three hours after its development, without impairing the loco-regional or distal neuro-muscular function. Local physical anamnesis revealed no acute or chronic sequela of lymphedema. (a)double superior vena cava defect(b)no sign of increased right atrial pressure(c)minimum bicuspid valve prolapse, with uneventful hemodynamic consequences(d)significantly reduced diameter of the left versus the right external jugular vein(e)negative dynamic maneuverers to check for the thoracic outlet syndrome.Previous echo-cardiography and color-doppler ultrasounds of supra-aortic trunks evidenced mild tissue edema in proximity of the neuro-vascular fasciculus, with: After careful and deep clinical evaluation to collect the most significant and reliable diagnostic information, we further investigated the patient through the total body computed axial tomography (CT), focusing on the thorax and, mainly, the mediastinum, with and without contrast medium. Photo Dynamic Eye (PDE) ,14 was aTomographic imaging confirmed double superior cava vein disorder with normal empty of the right cava in the ipsilateral atrium, and anomalous outlet of the left cava in the enlarged coronary sinus. A 3-D reconstruction of CT sections See  clearly The overall profile of lymphatic collectors, and of lymph node chains of the left neck, axilla and upper limb, were investigated through PDE lymphography ,15. Fluoph nodes .The morphological abnormality does not seem, per se, to be able to explain the observed transitory edema in the left supraclavicular neck area. In addition, the lymphographic images of the left upper limb and of the axillary lymph nodes show a conserved lymphatic transit. The slowed transport of indocyanine green observed with PDE lymphography  indicateAlthough transitory, the observed swelling presents a documented evolution  towards Lymphatic compression may determine, on one hand, an increase in lymphatic viscous flow resistances and, on the other, the enlargement of upstream lymphatic segments. This latter event would, in turn, cause: (1) a significant decrease of contractile strength of lymphatic smooth muscles and an impaired lymphatic propulsion , and/or (a)acute extrinsic compression ,3,6 at t(b)clavicular rotation itself, at a given decubitus, and, last but not least,(c)intrinsic venous maneuvers of self-squirm, upon movements of upper limbs or neck lateral rotation.The sequence of event described above may be exacerbated by patient posture during:In absence of severe disease symptoms, no therapeutic indication was provided, only suggesting specific postural adjustments of upper body segments. Edema worsening might eventually be treated with a multidisciplinary approach. At the four-year follow-up from our initial patient care, this advice helped the patient to successfully prevent the severity and discomfort of the occasional edematous events. In case of clinically worsening lymphedema, one might consider the advantages offered by the recently developed lymphatic vessels microsurgery ,13,14. T(a)manual lympho-drainage, consisting of superficial massages of edematous tissues to accelerate lymph transport towards the lymphatic network of healthy tissues. These maneuvers restore the drainage of the lymphedematous limb by recruiting collateral lymphatic vessels and/or shunts, able to vicariate the function of the armpit or groin lymph nodes resected, for example, during lymphadenectomy. In the specific case of post-mastectomy lymphedema, the attempt is to propel the lymph towards the terminus, the parasternal, paravertebral, contralateral axillary, ipsilateral inguinal and intercostal lymph nodes. Manual lympho-drainage presents few absolute contraindications, such as: (a) breasts’ neoplasm with systemic secondary localizations not surgically treated, or treated only with chemotherapy; (b) acute infections, inflammations and thrombosis, in which lympho-drainage could mobilize the limb thrombus, leading to pulmonary embolism or central nervous system stroke. Relative contraindications, in which lympho-drainage may be performed with adequate precautions, are, instead, pathologies such as systemic edema of cardiac or renal origin, or chronic infection, such as tuberculosis or toxoplasmosis;(b)compression garments, acting as a customed semirigid scaffold, exerting a progressively lower distal-to-proximal compression onto the lymphedematous limb . While t(c)physical exercise, possibly performed from the first postoperative days, to prevent development of post-surgically edema, optimizing the effect of manual lymphodrainage, and of compressive bandage. Exercises include correct breathing, stretching, self-mobilization and muscular strengthening;(d)intermittent pneumatic compression, or pressotherapy, is performed through a cuff applied to the lymphedematous limb and pressurized according to specifically costumed clinical protocols under close supervision of specialized health personnel. It represents an efficient therapy when accompanied by lymphodrainage and compressive hosiery, but it cannot replace them. Pressotherapy presents the same contraindications as manual lymphodrainage.The target of physical therapy is to improve lymphedematous tissue drainage by enhancing local lymph propulsion, and by recruiting shunted pre-existing lymphatic vessels. If lymphedema is localized in the upper or lower limbs, a possible approach consists of the so-called Complex Decongestive Therapy, including manual lympho-drainage, garments, physical exercise, skin care and prevention . Therapy(a)use of a compressive-elastic brace, a crucial element of the therapy, with a function similar to compressive garments;(b)accurate skin care, to prevent ulcerations and frequent complications, such as development of erysipelas, fever, itching, skin erythema, limb heat and pain. In addition, it is of absolute importance to wear the compressive garments prescribed at the hospital discharge.If limb volume is satisfactorily decreased and stable, one can move to the maintenance phase, implying:In case physical therapy does not satisfactorily reduce lymphedema and/or the degree of patient discomfort, one may consider the opportunity of looking to a specific surgical approach. The latter exploits various procedures aimed at recanalizing the lymphatic ducts (lympho-lymphatic shunt), whose pathway has been obstructed, and/or at realizing a low resistance inlet of a lymphatic vessel directly into a peripheral vein (lympho-venous shunt).Microsurgery may be described as a multidisciplinary branch of surgery, derived from experiences developed on laboratory animals to approach tiny organic structures with dimensions in the order of microns; it is performed through the aid of an intraoperative microscope, microsurgical instruments, and suture stitches from 9–0 to 11–0.In the last 50 years of the past century, the development of manufactory industries was accompanied by a parallel increase of the occurrence of serious blunt trauma in workplaces, with increasing episodes of limb and/or finger amputations. In addition, the increased use of motorbikes, especially among young people, sometimes driven at exceedingly high velocities, determined the increase of serious road accidents, resulting in severe lesions of the nervous system  and/or of the bone-muscle-joints complexes, with involvement of both large vascular trunks and microvasculature of limbs or extremities. To face the increased need of new strategies for the treatment of these complex clinical conditions, either in emergency or as a specific focused service, new dedicated Trauma Centers developed, with the aim of offering an adequate and modern multidisciplinary approach to enable the patients to recover their total functional integrity and be fully reinstated into society. In parallel, experimental studies on laboratory animals involved an increasing number of surgical areas: from general surgery to orthopedics, neurosurgery, vascular and thoracic surgery, gynecology, and from experimental organ transplants to ophthalmological surgery. In this context, the first microsurgical artero-arterial , veno-venous  and nervous  anastomosis were perfected in rats. In the following years, to develop new approaches able to avoid palliative demolitive interventions in the treatment of peripheral lymphedema, surgeons started focusing their attention on the fine anatomy, the physiology, and the physiopathology of the vascular lymphatic system. New techniques were perfected in laboratories to set up all types  of lympho-venous anastomosis . In addi++-antagonists.Preoperative screening for microsurgery of lymphedema includes, in addition to the feedback of the physical examination and imaging diagnostics, the data collection of medical history, including (a) remote, recent and family objective cardiovascular and neurological pathology, (b) allergies, habits and diet, and (c) current medical therapies, in particular, on the use of CaMicrosurgery of the upper or lower limbs requires lymphoscintigraphy and a color doppler ultrasound, to rule out the concomitant occurrence of other vascular diseases, such as, for example, deep vein thrombosis. Instead, lymphedema of external male genital organs can be instrumentally diagnosed only through echography. The following sections illustrate the current microsurgical approaches and their long-term outcomes.This procedure is usually performed to solve cases of secondary lymphedema of the upper limbs, but it may also be applied to lower limbs. First, one must proceed with the identification of the major lymphatic collector of the healthy lower limb. With the patient deeply anesthetized, 1 mL of Patent Blue dye is injected in the 2nd interdigital space of the same limb. Under stereomicroscopic view, the lymphatic collector is identified and excised , while pAfter setting up and identifying the lymphatic-lymph node structures of the supraclavicular-ipsilateral area, and of the upper third of the medial arm in the proximal axillary area, the lymphatic graft is then inserted though axillary subcutaneous tunneling , allowinThe surgical approach to identify the lymphatic collectors and venous vessels of upper and lower limbs and genitalia is identical. In lower limbs, super-microsurgery protocol  allows fUnder general anesthesia, following inguinoscrotal, cremaster and vaginal tunica incisions, the funicular lymph vessels (diameter ≈500 mm) and cord veins can be observed bilaterally under stereomicroscopic view. These collectors are used to perform either lymphovenous termino-lateral or termino-terminal anastomosis, depending upon the proximity and pathway of the pampiniform veins, which discharge in the ipsilateral spermatic vein, and of the spermatic lymphatic collectors. After removal of vein adventitia , followiIn the lower limb, the lymphatic system is organized in: (a) a superficial network, merging into lymphatic collectors directed to the inguinal lymph nodes, from which the lymph is carried through the iliac collectors into the cisterna chili and, thereafter, to the thoracic duct and, eventually, into the subclavian veins; (b) a deeper network, whose collectors follow the sub-fascial limb neuro-vascular plexus to reach the peri-aortic lymph nodes and the thoracic duct.Lymphedema of male external genitals is often accompanied by unilateral or bilateral chronic swelling of the lower limbs. As such, the microsurgical approach consists of a two-stage treatment: first, genital microsurgery, and, after six months, if needed, lympho-venous shunt of the lower limb .The results of this approach clearly indicate that the shunt of the spermatic cord in the treatment of genital edema was often accompanied by a significant improvement of the edema of the lower limbs, such as to lead us, after a few cases, to subsequently exclude the two-step surgery. Improvement of limb lymphedema after shunt of the spermatic cord may depend upon the fact that, after excision of inguinal lymph nodes, lymph from lower limbs is shunted through the scrotal and testicular pathways. Similarly, when testicular drainage is restored, edema of the scrotum and penis is also significantly drained, possibly through direct connections between scrotal lymphatics and testicular networks.A positive long-term postsurgical recovery of the patient critically depends upon the maintenance of anastomosis patency. The degree of recovery at 180 days from surgery in subjects treated for arm or lower limb lymphedema may be examined using: (a) noninvasive PDE lymphography (upper limb, Patients can be considered “responders” if, at 6 months after surgery, limb volume decreased by >60% of the preoperative lymphedematous volume.In a previous study from our Lymphedema Center , in patiAt 180 days from surgery, absolute limb volume decreased by 35% in responding patients, with excess limb volume decreasing by about 120% L. A sligIn those with lower limb lymphedema, the swelling increased by ≈40%, with respect to the other normal limb L. At 180About 70% of patients undergoing microsurgery of the spermatic cord attained a 95% 100% scrotal swelling remission at 6 months after surgery . In all A set of patients only partially improved after the procedure, with ≈60% assessment at 180 days from surgery.In addition to maintenance of patent lymphatic collectors, a common observation in responding patients is the development of complex lymphatic meshes, well documented by PDE F, and/orLymphatic meshes did not apparently develop in nonresponding patients, nor in the area around lymphatic grafting when the anastomosis was confectioned with two biologically similar tissues, such as terminal stumps of lymphatic collectors. This clinically relevant phenomenon requires further investigation to clarify the biologic processes of acute lymphangiogenesis after tissue trauma or surgery. Indeed, at present, it is not clear whether these new vessels reflect passive recruitment of previously not utilized vessels or new active sprouting.In several tissues, lymphatic vessels may merge into networks shaped like closed loops in the diaphragm ,23, or aLymphedema is a complex pathology of the lymphatic system that, regardless of its origin, may present different clinical manifestations. Physical therapy must be tried, at first, in the treatment of both primary and secondary lymphedema; only after its failure, other procedures must be considered. Particularly, in cases of secondary lymphedema, the bio-technological advances and/or refinement of more and more accurate surgical instruments, as well as the development of new surgical procedures, allowed for the attainment of satisfactorily results, in line with the required clinical criteria. Indeed, with respect to the previously widely utilized demolitive oncological interventions, the microsurgical approach minimized the relative sequelae or iatrogeneses. Interestingly, in patients successfully treated with microsurgery of spermatic and lower limb lymphatics, new lymphatic meshes develop to optimize perianastomotic tissue drainage. Hence, at variance with more destructive surgery, minimally invasive microsurgery might promote local neo-lymphangiogenesis. Therefore, more research studies are needed to further refine the bioengineering development associated to microsurgical approaches that not only reduce the patient hospitalization and recovery period, but seem to facilitate, with respect to more invasive surgical procedures, the lymphatic and extracellular matrix fibrous tissue repair.


Cholesterol crystal embolism is a rare microembolic disease caused by cholesterol crystals that can present with various symptoms after vascular surgery, catheterization, or anticoagulation therapy. We report a case of penile ulceration caused by cholesterol crystal embolism.A 72‐year‐old man undergoing maintenance dialysis for end‐stage renal failure presented with penile pain and a black glans ulcer. Despite low‐density lipoprotein apheresis, he was referred to our hospital because of lack of improvement. Based on his medical history and clinical presentation, including artificial vascular replacement and right toe amputation, cholesterol crystal embolism was suspected and partial penectomy was performed, thus confirming the diagnosis. Penile pain resolved after surgery, and he was discharged on Day 10. Unfortunately, he died after small bowel perforation developed 2 months after surgery.Penile ulcers caused by cholesterol crystal embolism may indicate the severity and progression of disease and typically require surgical intervention. Cholesterol crystal embolism‐induced penile ulcers require early consideration as the differential diagnosis and often require surgical intervention. Conservative treatment is usually ineffective. It is important to improve outcomes through case accumulation. These ulcers may reflect the disease severity and prognosis.AAaortic aneurysmAAAabdominal aortic aneurysmAFatrial fibrillationASaortic stenosisASOarteriosclerosis obliteransCAGcoronary angiographyCCEcholesterol crystal embolismDLdyslipidemiaDMdiabetes mellitusEVARendovascular aortic repairHDhemodialysisHThypertensionLDLlow‐density lipoproteinMRmitral regurgitationPFprecipitating factorCCE is a systemic microembolic disease triggered by cholesterol crystals that can present with various symptoms and is often associated with major vascular surgery, intravascular catheterization, or anticoagulation therapy.A 72‐year‐old man with a history of chronic renal failure who was undergoing maintenance dialysis for end‐stage renal failure presented with penile pain and a black ulcer on the glans penis. His medical history included chronic renal failure, acute myocardial infarction, AAA, bilateral toe gangrene, small bowel perforation, HT, and DL. He had previously undergone percutaneous coronary intervention for acute myocardial infarction, artificial vascular replacement surgery for left iliac artery stenosis, and right toe amputation because of CCE. Subsequent repeated gangrene development in his toes required multiple amputations. Dual antiplatelet therapy was required for 6 months after acute myocardial infarction. Thereafter, single‐agent antiplatelet therapy was required. Despite LDL apheresis for penile pain, he was referred to our hospital because of lack of improvement.A black ulcer measuring 10 mm on the glans penis was observed during the physical examination Fig. . LaboratAlthough malignancy was not ruled out completely as the differential diagnosis, CCE and other conditions that cause penile ulcers, such as calciphylaxis, were strongly suspected based on his medical history and clinical findings. However, because of his severe penile pain, conservative treatment was not an option. Therefore, we explained the risks and benefits to patient and decided to perform a partial penectomy. We did not perform a histopathological examination to determine the tissue diagnosis.During partial penectomy, an adequate margin of approximately 3 cm around the ulcer was ensured to allow for the preservation of approximately 50% of the penile tissue. A pathological examination revealed epidermal detachment, necrotic tissue adherence, and inflammatory exudate in the ulcer. Moreover, cholesterol clefts with thrombotic occlusions were observed in the surrounding vessels, and the diagnosis of CCE was confirmed Fig. .After surgery, the penile pain gradually decreased and eventually disappeared. Despite the presence of hypoalbuminemia, the postoperative course was relatively favorable. The patient was discharged on Day 10. However, he died after the development of small bowel perforation 2 months after surgery.CCE is mainly caused by the rupture of atheromatous plaques in large arteries, such as the abdominal aorta, iliac artery, and femoral artery, which leads to the occlusion of small peripheral arteries by cholesterol crystals, resulting in thromboembolism in multiple organs.CCE‐induced penile ulcers are rare; including the present case, only 10 cases have been reported (Table CCE‐induced penile ulcers are believed to result from thrombosis in the peripheral artery of the penis, which branches from the internal pudendal artery.CCE‐induced penile ulcers may reflect the severity and progression of disease and often require surgical intervention.Toshiki Matsushima: Writing – original draft. Kensuke Mitsunari: Project administration; supervision; writing – original draft; writing – review and editing. Nobuhide Maekawa: Writing – review and editing. Shota Yamada: Writing – review and editing. Hidenori Ito: Writing – review and editing. Hiroki Kurata: Writing – review and editing. Yuichiro Nakamura: Writing – review and editing. Tomohiro Matsuo: Writing – review and editing. Kojiro Ohba: Writing – review and editing. Ryoichi Imamura: Supervision; writing – review and editing.The authors declare no conflict of interest.No ethics approval was required for this case report.Written informed consent was obtained from the patient.Not applicable.


Hepatic small vessel neoplasm (HSVN) is a recently described vascular neoplasm of the liver. It demonstrates an infiltrative growth pattern and lacks cytologic atypia and mitotic activity. So far, no cases of metastasis or disease recurrence after excision have been reported in the literature. In this report, we present the case of a 31-year-old woman with a lesion in segments VII-VIII of the liver who was referred to our surgical department due to right lumbar pain. She underwent an atypical wedge hepatectomy  and cholecystectomy. The histopathology of the resected specimen confirmed a 40mm HSVN. The patient did not receive any adjuvant therapy and is scheduled for follow-up with serial magnetic resonance imaging (MRI) scans over the next five years due to the unknown malignant potential of the tumor. Hepatic vascular tumors cover a wide spectrum of benign and malignant lesions ranging from the common cavernous hemangioma to the rare and aggressive hepatic angiosarcoma. This category of liver tumors also includes Kaposi's sarcoma, hemangiopericytoma, epithelioid hemangioendothelioma, and the recently described hepatic small vessel neoplasm (HSVN) .HSVN is a low-grade vascular neoplasm of undetermined malignant potential, first described by Gill et al. in 2016 . In the  A 31-year-old woman was referred to our department with a six-months history of pain in the right lumbar area. The patient's medical history and physical examination of the chest and abdomen were unremarkable. Chest X-ray also came back normal. Hematological and liver function tests were within normal ranges. A contrast-enhanced computed tomography (CT) scan showed an inhomogeneous lesion of approximately 5.7x4.7cm in segments VII-VIII of the right lobe of the liver. The lesion exhibited strong contrast enhancement in the arterial phase, remained strongly hyperdense in the portal venous phase, and became isodense in the late acquisitions (pooling). It was surrounded by a halo and showed multiple arterial feeding vessels. In the MRI series, the lesion exhibited a low signal on T1 sequences and a high signal, along with the presence of a central area with a low signal on T2 sequences , contrast-enhanced ultrasound (CEUS) showed in the arterial phase (13 sec) a strong uptake from the periphery to the center with complete uptake at 20 sec  Figure  and cholThe resected specimen was a vascular neoplasm measuring 4cm at its maximum diameter, mostly consisting of small-diameter, thin-walled vascular spaces lined by oval cells of endothelial origin. The tumor developed infiltratively between the hepatic trabeculae and enclosed portal spaces and interlobular cholangia, extending at a great distance from the main tumor, focally reaching its surgical border. Papillary formation, layering, nuclear pleomorphism, mitotic activity, or necrosis of neoplastic cells were not observed . The CAMTA1 marker for epithelioid hemangioendothelioma, as well as staining for c-Myc and p53, were negative. The cell proliferation index Ki67 was difficult to calculate due to lymphocytic infiltration, but it was estimated to be less than 10%. These findings were consistent with a hepatic small vessel neoplasm. The patient is currently scheduled for an initial follow-up with CEUS and MRI six months after resection, with close surveillance planned for the next five years.Hepatic small vessel neoplasm is a vascular tumor of the liver that has been recently recognized. However, its histopathology and clinical behavior have not been adequately established in the medical literature. Its true incidence and long-term prognosis remain unclear, and case reports and series present diverse histopathological descriptions, utilize different imaging modalities, and focus on varied aspects of this pathological entity. To our knowledge, there have been 25 cases Table  detectedAlthough HSVN exact etiology is still vague, the activating hotspot of GNAQ mutation seems to be an important step in HSVN tumorigenesis and malignant potential . The rolClinical presentation of patients with HSVN is often occult, with the majority of the lesions detected as incidental findings. Patients may complain of fullness, abdominal pain, or fatigue or exhibit abnormal liver function tests. Our patient was referred to our surgical department with a history of right lumbar pain lasting for six months.On radiological imaging, HSVN displays an early peripheral continuous enhancement with persistent enhancement in portal venous and late phases . These fAs far as morphologic features are concerned, hepatic small vessel neoplasm is an infiltrative tumor composed of thin-walled small vascular spaces lined by flat to plump-oval endothelial cells. The cells comprising the lesion lack features of cytologic atypia, such as mitotic activity, necrosis, or nuclear irregularity/pleomorphism . ImmunohIn our case, the diagnostic workup included blood and liver function tests, a CT scan and MRI of the abdomen, as well as contrast-enhanced ultrasound, which raised the suspicion of HSVN. We performed an atypical wedge hepatectomy  and a cholecystectomy under intraoperative ultrasound guidance.We noticed that, after the disruption of its vascular supply, the tumor decreased in size, making it difficult to distinguish from normal liver parenchyma. Thus, we highlight the importance of preoperative imaging and surgical planning, as well as the use of intraoperative ultrasound to locate the lesion and ensure negative resection margins.Furthermore, we propose that all cases of suspected or diagnosed HSVN be referred to tertiary hospitals with a specialized hepato-pancreato-biliary (HPB) unit. We underline the importance of a multidisciplinary team (MDT) approach involving an oncologist, surgeon, diagnostic and interventional radiologist, hepatologist, pathologist, endoscopist, and gastroenterologist, given the rarity of these newly described tumors.At present, more data and case series are required to establish safe diagnostic criteria and therapeutic guidelines for hepatic small vessel neoplasms. Therefore, we suggest tumor resection followed by close and long-term follow-up for these patients.HSVN, or hepatic small vessel neoplasm, is a vascular neoplasm of the liver characterized by an infiltrative growth pattern. Based on the currently available data, it appears to have a benign clinical course. More research and case series are essential for establishing reliable diagnostic criteria as well as therapeutic and follow-up guidelines.


Plexiform schwannomas are rare, benign, neural crest-derived tumors that commonly occur in the hand and upper extremities. They may be sporadic or associated with neurofibromatosis type 2. Although previous literature has described plexiform schwannomas occurring in fingers, nerve and tendon sheaths, and intraosseous lesions, this is the first known case of a plexiform schwannoma of the thumb. This is a case of a growing, painless, subungual mass of the thumb in a 54-year-old patient. After surgical excision and subsequent immunohistochemical examination, the patient was diagnosed with a plexiform schwannoma. This highlights the importance of maintenance a broad differential before surgery and obtaining a proper diagnosis using histopathology. Schwannomas, also known as neurilemmomas, are benign, neural crest-derived proliferations of peripheral nerve sheath cells. These masses account for approximately 5% of all tumors involved with the upper extremities and 2.8% of biopsied benign, soft-tissue tumors of the hand and wrist.This report describes a rare case of a plexiform schwannoma of the volar aspect of the thumb in a 54-year-old patient. After surgical excision and subsequent immunohistochemical examination, the patient was diagnosed with a plexiform schwannoma. Written informed consent was obtained from the patient for publication of this case report and accompanying images.A 54-year-old woman with a medical history of chronic kidney disease stage 2 and prediabetes mellitus presented for the evaluation of a solitary, painless mass over the subungual region of her right thumb. The mass had been growing for approximately 7 years but doubled in size over the past year, resulting in a notable deformity. Because the patient was dissatisfied with the cosmetic appearance of her thumb, she requested surgical excision. Radiographs were obtained and magnetic resonance imaging was performed to assess for scalloping of the distal phalanx. The radiographs of the thumb were unremarkable. Magnetic resonance imaging showed a septated, large volar mass along the distal phalanx with low intensity on T1 and high intensity on fluid sensitive sequences . After gThe patient was brought to the operating room and placed in a supine position. After the patient was placed under general anesthesia, the right upper extremity was prepared and draped in the usual sterile fashion. The upper extremity was exsanguinated using an Esmarch bandage . The patient had a prosthetic nail in place, which was trimmed back in order to gain access to the hyponychium. A fishmouth-style incision was made around the tip of the thumb, after which the skin over the lesion was peeled back. The lesion appeared to be soft and well circumscribed. It was gray and white in color and approximately 2 cm in diameter. The entire mass was excised in 1 piece and sent to the pathology department for review . Under lThe surgical pathology department examined the excised specimen and identified a well-circumscribed tumor with a myxoid background and foci of microcytic changes as well as wavy, slender nuclei with tapering ends, suggestive of neural differentiation . The tumAfter surgery, the patient’s incision healed well and the thumb remained neurovascularly intact , 6. She ,,Plexiform schwannomas are solitary, benign, peripheral nerve sheath tumors that typically localize in nerve plexuses, the skin, and subcutaneous tissues of the upper and lower extremities.Plexiform schwannomas are not visible on radiographs but have a hyperintense signal on T2 on magnetic resonance imaging, as seen in this case.,The immunohistochemical markers used to detect plexiform schwannomas include S-100 , calretinin or CD56, SMA, and desmin.This case was unusual given the location of the plexiform schwannoma in the thumb. This highlights the importance of maintenance of a broad differential before surgery and obtaining a proper diagnosis using histopathology. The diagnosis of 1 lesion may help guide management for future lesions found later . Ultimately, histologic assessment is required for definitive diagnosis of plexiform schwannomas.


Desmoid fibromatosis is a rare benign neoplasm of the soft tissue. Primary desmoid neoplasms rarely occur in the small bowel and are primarily found in patients with a previous abdominal surgery or irradiation history. They are challenging to diagnose at the time of presentation due to a lower incidence and their non-specific presentation making it difficult to distinguish from other intra-abdominal neoplasms, such as gastrointestinal stromal tumors (GISTs), which may present with similar symptoms. We like to present a case of a 34-year-old male with a four-day history of abdominal pain with worsening severity and one episode of non-bloody vomiting. Physical examination was significant for generalized abdominal tenderness with positive rebound and board-like rigidity. A computed tomography (CT) scan of the abdomen showed the presence of a lower abdominal mass of unknown etiology with free air foci and free intraperitoneal fluid either due to rupture of the suspicious mass or secondary to infection by an air-producing organism. The patient was immediately taken for emergency surgery, the tumor was resected successfully, and a specimen collected was sent for histopathology, which came out to be a desmoid tumor. We aim to highlight the importance of keeping a broad differential diagnosis in a patient with acute abdomen and symptoms of peritonitis. Desmoid tumors are locally aggressive but benign soft tissue tumors originating from the mesenchymal cells with a high recurrence rate but without the propensity to metastasize . The estA 34-year-old male presented to the emergency department with abdominal pain that started in the periumbilical area and later migrated to the right iliac fossa with one episode of non-bloody vomiting and a fever of four days duration. Past surgical history was significant for an open inguinal hernia repair done seven years back. On physical examination, the patient was febrile, tachycardic, and ill-appearing. Abdominal examination was significant for generalized abdomen tenderness with rebound and board-like rigidity. The rest of the physical examination was otherwise unremarkable. A working diagnosis of perforated appendicitis was made.An erect X-ray of the chest and abdomen was done to rule out perforated peptic ulcer disease. The X-ray abdomen showed the presence of few air-fluid levels. Then the decision to proceed with a CT abdomen was made, which showed a large mass with a hypodense center and a marginal enhanced shaggy wall seen at the center of the lower abdomen, inseparable from the nearby small bowel loops Figure . Also, mThe patient underwent diagnostic laparoscopy, which showed the presence of a large lower intra-abdominal mass arising close to the distal ileal mesentery root with the involvement of a segment of the ileum loop. The procedure was converted to laparotomy, which showed a sizable perforated mass of around 10 cm, arising close to the root of mesentery of the distal ileum Figure , involviThe peritoneal fluid was sent for microscopy, revealing scattered mesothelial cells in a background composed of neutrophils, histiocytes, lymphocytes, and occasional plasma cells. However, no component of malignant cells was seen from the sample obtained. The histology report of the collected sample showed an intestinal spindle cell tumor with capsular rupture, partial infarction, ischemic changes, and involvement of the mesenteric margin. The lateral margins of resection were free of the lesion. The pathological slides reported features suggestive of desmoid fibromatosis Figures , 4B.Immunohistochemical stains were performed and were strongly positive for β-catenin and vimentin Figure . Other pDesmoid neoplasms are infrequent and comprise 0.03% of all neoplasms and 3% of soft tissue neoplasms in general . Extra-aDesmoid tumors are benign mesenchymal tumors that exhibit fibroblastic cells embedded in a stroma of collagen and spindle cells in histology. According to several studies, there is a higher incidence of desmoid tumors in female patients, especially those with a history of oral contraceptive pills and during gestation and postpartum, which is in contrast to the male patient in this case . A priorIt has also been reported to occur due to the intranuclear accumulation of β-catenin, which has been found to occur due to mutations in the Wnt/β-catenin gene. Immunostaining markers of β-catenin have, in recent times, immensely helped in detecting antigens that can assist in differentiating between desmoid neoplasms and other fibroblastic neoplasms that may have similar findings on gross appearance and histology . Our patIntra-abdominal tumors usually do not manifest with any symptoms in the early stages. As a result, the commonly encountered signs and symptoms of abdominal pain, vomiting, gastrointestinal bleeding, or a palpable mass are usually not detected until late in the course of the disease. The complications that may be commonly encountered due to intra-abdominal tumors include entero-cutaneous fistulae, intestinal perforation, intestinal hemorrhage, bowel obstruction, and ureteric obstruction . Our patDue to the uncertain etiology and life-threatening condition, a surgical approach for management had been planned in our case. However, research articles have found that different options can be employed to manage patients with desmoid tumors. Chemotherapy, radiotherapy, and antihormonal therapy with nonsteroidal anti-inflammatory drugs are successful. It is encouraged to manage such tumors meticulously by starting with less toxic medications and then escalating to more potent drugs if necessary. A “watch and wait strategy” is proposed for patients, and active intervention is only recommended in patients with a progressive course or acute symptoms.  On the oIn view of the relative rarity of the incidence of the neoplasm intra-abdominally, there has been difficulty in establishing a standard plan of care. It may be challenging to differentiate from other neoplasms that present similarly, such as GIST; hence, there is a need for biopsy and further immuno-staining and histology. Desmoid fibromatosis may present with atypical signs and should be included when evaluating patients with peritonitis.


Glioblastoma (GBM) is the most common brain tumor and has a median survival of less than two years. The current standard of care consists of surgery followed by concomitant radio and chemotherapy with temozolomide. Although it is an aggressive tumor, distant metastases are extremely rare. The dissemination mechanisms are not fully understood and currently there is no standard of care for its treatment. In this study, the authors present a comprehensive analysis of all the existing cases of extra-neural metastases of GBM in a tertiary care center over the last five years, along with the procedures carried out in each case. Glioblastoma (GBM) is the most common malignant brain tumor, making up 46.1% of all primary brain tumors . It has This study aimed to report all cases of extra-neural metastases of GBM that were diagnosed in the University Hospital Center of São João since 2017 in order to increase physicians' awareness of this rare neoplasm and its treatment approaches in clinical practice.From January 2017 to December 2021, 314 cases of GBM were diagnosed in adults in our center, three of which had extra-neural metastases (0.95%).Case 1A 75-year-old female presented with sudden decreased muscle strength in the left arm. She resorted to the emergency service and a head computed tomography (CT) was immediately made, describing a lesion compatible with a malignant tumor. A cerebral magnetic resonance imaging (MRI) was performed to clarify these findings and it showed a large expansive lesion in the right frontal region measuring 58 × 38 × 50 mm, which can be seen in Figure 2, followed by temozolomide 150 mg/m2 for five days every 28 days) [The tumor was subjected to complete resection of the lesion and the anatomopathological (AP) report revealed a GBM isocitrate dehydrogenase (IDH) wildtype without methylguanine methyltransferase (MGMT) gene promoter methylation. She was treated with concomitant radiotherapy (RT) and chemotherapy according to the modified Stupp regimen  [The patient was submitted to surgery and the post-operative MRI showed tumor residue, therefore it was considered an incomplete resection; the AP report indicated that it was an IDH-wildtype GBM. Consequently, the patient initiated the Stupp protocol (60 Gy administered in 2.0 Gy fractions over six weeks with concomitant temozolomide 75 mg/mAfter the seventh cycle of adjuvant temozolomide, the patient was admitted to the hospital due to large volume pleural effusion causing dyspnea and heavy coughing. The patient was admitted to the inpatient clinic and the physicians proceeded with the investigation of what was thought to be another primary tumor. In the etiological study that was carried out, the CT highlighted the thickening of the pleural leaflets, multiple metastatic pulmonary nodules, and four hepatic nodules Figures , 4. The pleural fluid anatomopathology was negative for malignant cells and no alterations were found in the mammography, thyroid ultrasound, endoscopy, and colonoscopy. The liver biopsy, scheduled for later, revealed GBM metastasis Figure . RegardiDuring hospitalization the patient had cognitive and neurological deterioration, thus it was decided for best supportive care strategy; given that the patient was not submitted to any other invasive techniques, the pulmonary metastases were never confirmed. She passed away seven days after confirmation of metastasis.Case 3A 64-year-old male had gradual muscle strength loss in the upper and lower left limbs and left central facial paresis for roughly six weeks. The first examination that was made was a head CT, and based on the findings, he was subjected to an MRI, which showed a right frontal lesion of 4 cm, highly suspicious of a high-grade glioma (Figure He underwent complete macroscopic resection of the lesion and the anatomopathological report revealed an IDH-wildtype GBM. The patient recovered from focal deficits after surgery and initiated treatment with Stupp protocol. After the first cycle of adjuvant temozolomide the patient had two generalized tonic-clonic seizures and the MRI displayed signs of disease progression. Therefore, he initiated second-line chemotherapy with lomustine and bevacizumab approximately seven months after the surgery. After two cycles, swelling in the posterior region of the craniotomy was clinically observed. The cerebral MRI presented an expansive right parietal epicranial lesion Figure , whose eThere was no evidence of progression regarding the primary cerebral lesion. Therefore, the patient resumed treatment with bevacizumab and lomustine and started external RT to the scalp with palliative intent, with a total dose of 20 Gy, at 4 Gy per day, in five fractions, with 9 MeV electron energy. He died five months later, with disease progression.Despite the medical advances regarding multimodality treatments with chemotherapy and RT, GBM bears an overall poor prognosis, with a median overall survival of 15 months with optimal treatment . In addiThere have been more documented cases of extra-neural metastases of GBM in the last few years. However, this number remains low due to the reduced survival rate, only some cases have time to disseminate. Furthermore, some of the described case reports were post-mortem findings, not displaying any signs or symptoms during lifespan -12,14. TRegarding the anatomopathological findings which characterize GBM, there are more densely cellular regions, with greater nuclear pleomorphism, several mitotic figures, necrosis, and vascular and endothelial cell proliferation .The pathophysiological mechanisms of the systemic dissemination of GBM are still not completely clarified. One of which is the breaking of the blood-brain barrier, either by the tumor itself or the surgical procedure, permitting access to the blood and lymphatic vessels. The subcutaneous metastasis of the third case could be justified by the surgical approach, given the location. This mechanism appears to be related to a dissemination process and not exactly by distant metastasis. Other described mechanisms are the invasiveness of the tumor, resistance to the immunologic defense mechanisms, and adaptation to hostile environments through quiescence . All of The proportion of cases in this hospital and the survival rates after the diagnosis of metastases are congruent with existing literature, with the exception of the last case, whose mechanism seems to have been by dissemination after the surgical procedure and not distant metastasis. The diagnosis of extra-neural metastization of GBM provides a dismal prognosis, for which there is still no standardized treatment. The pathophysiological mechanisms of this systemic dissemination are quite unclear and may be useful in the development of appropriate therapy. This, along with the early referral to palliative care will bring not only more adequate treatment options and increase survival outcomes but also improve the quality of life of both patients and caregivers.


De-tubularised ileum is one of the most common segments used for augmentation cystoplasty. It is associated with complications such as metabolic disturbances, recurrent urinary tract infections, and stone formation. However, adenocarcinoma arising in an augmented bladder is a rare occurrence. We report a 37-year-old female, case of ileocystoplasty 25 years ago due to a thimble bladder (genitourinary tuberculosis) who presented with hematuria for one month. Cystoscopy showed bladder mass in the transposed ileal segments. The patient underwent transurethral resection of the bladder lesion, and the histopathology was suggestive of adenocarcinoma of the ileum. Subsequently, she underwent anterior pelvic exenteration and post-operative recovery was uneventful. The 6-month follow-up showed that the patient was asymptomatic without recurrence. In conclusion, even though adenocarcinoma in the ileal neobladder is rare, life-long with close follow-up with routine cytologic, radiologic, and cystoscopic evaluation for early cancer detection and treatment at an early stage is crucial. In tubercular small-capacity bladders, augmentation cystoplasty is usually done when nonsurgical management has failed . VariousPatient information: a 37-year-old lady presented with complaints of painless gross hematuria and dysuria for one month. In past, she had undergone augmentation ileocystoplasty at 12 years of age for a thimble bladder and left simple nephrectomy for pyonephrosis due to genitourinary tuberculosis. One year back she underwent a cystoscopy at an outside center for similar complaints that showed a tumor lesion measuring 1 x 1 cm and located at the bladder dome. The tumor was completely ablated endoscopically. There was no histopathology or urine cytology report available from that event. The patient did not have any associated chronic medical illness, or history of any substance abuse, or addiction. There was no family history of similar complaints.Clinical findings: the patient´s vital signs and abdominal exam were normal.Diagnostic assessment: her blood investigations revealed hemoglobin of 12.4 g/dl and serum creatinine of 2.5 mg/dl. Liver function tests and serum electrolytes were in the normal range. Non-contrast computed tomography of the kidney, ureter, and bladder region (NCCT-KUB) revealed mild hydronephrosis and complete mild hydroureter of the right side. The urinary bladder was distended and showed a lobulated contour. There was an irregular, lobulated, enhancing soft tissue lesion along the anterior vesical wall in the transposed ileal segment measuring 2.1 x 4.7 x 4.8 cm with mild peri vesical fat stranding. There was no evidence of any enlarged lymph nodes on NCCT-KUB  under general anesthesia. On cystoscopy, there was a solid proliferative growth along the anterior vesical wall in the transposed ileal segment measuring 2 x 5 x 5 cm with evidence of neovascularization. The right ureteric orifice was not visible. The biopsy report was suggestive of adenocarcinoma of the ileum. The patient subsequently underwent anterior pelvic exenteration , and right-end ureterostomy . Flourine-18 fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) at 3 months postoperatively showed no evidence of abnormal uptake or suspicious metabolic active disease. At 6 months follow-up, the patient was asymptomatic with a serum creatinine of 2.2 mg/dl. A medical oncologist´s opinion was taken. The patient did not receive any adjuvant treatment in view of the absence of lymph nodal involvement and the absence of any metabolic active disease on FDG PET/CT.Patient perspective: during her hospitalization and after treatment, the patient was satisfied with the care she received.Informed consent: written informed consent was obtained from the patient for participation in our study.et al. suggested that there is a 7-8-fold increased risk of malignancy following an ileal or colonic augmentation and 14-15-fold following gastric augmentation [et al. found no significant difference in the incidence of cancer between the three different types of bowel segments used [Various types of gastro-enteric segments are used for augmentation of the small-capacity bladder. The ileum segment is the most common bowel segment used. The probability of developing a malignant tumor post-augmentation cystoplasty ranges from 0 to 5.5% . Some auentation . Howevernts used .The carcinogenesis in the augmented bladder can be attributed to various mechanisms. Chronic inflammation because of bacteriuria is one factor. It results in the release of fibroblast growth factors, prostaglandin, eicosanoids, cytokines, and free oxygen radicals which can lead to carcinogenesis . Secondlet al. reported 2 cases of adenocarcinoma that developed in augmented bladders 23 and 30 years after ileocystoplasty, and both tumors were associated with contracted bladder due to tuberculosis [et al. reported that in the majority (50%) of the cases, a tumor arises at the entero-urinary anastomosis [Although adenocarcinoma is the most common histology found in augmented bladders, varied histopathology has been reported, like urothelial carcinoma, squamous cell carcinoma, sarcomas, and small cell neuroendocrine tumors ,9. The lrculosis . Similarrculosis . Biardeaet al. pointed out that at present annual cystoscopy started 10 years after the surgery is the only reliable tool for surveillance [There are no recommended guidelines for surveillance ,10. Altheillance . So, it Even though adenocarcinoma in the augmented ileal neobladder is rare, life-long with close follow-up with routine cytologic, radiologic, and cystoscopic evaluation for early cancer detection and treatment at an early stage is crucial. Patients' education and regular follow-up at specialized centers where standard management protocols for such malignancies are established are highly recommended.


Occlusive hernias are rare and difficult to diagnose. We present an extraordinary case of simultaneous occurrence of an obturator hernia with colon cancer. An 86-year-old woman arrived at the hospital after ˃2 weeks of abdominal pain, nausea, vomiting, and constipation. The computed tomography axis map showed that part of the right lower abdominal small intestine had intruded into the femoral triangle through the obturator, which was diagnosed as an obturator hernia. When the abdominal cavity was opened for herniorrhaphy, a 4 × 4 cm colon mass was observed. Only herniorrhaphy was performed, without any complications. At present, there has been no report of the coexistence of occlusive hernia and colon cancer; the main symptoms are intestinal obstruction, nausea, vomiting, and constipation. The decision whether the tumor should be removed simultaneously with herniorrhaphy and/or a mesh patch. An obturator hernia is a protrusion of an organ/tissue through the obturator canal. It is commonly known as “little old lady’s hernia,” as they typically affect older, thin female patients . They arOne of the most aggressive types of cancer is colorectal or colon cancer. It is the third most common cancer and a significant cause of morbidity and mortality worldwide . We repoAn 86-year-old female complained of constipation with abdominal pain, nausea, and vomiting for ˃2 weeks. The patient had defecated once, after conservative treatment with an enema in a local hospital, with less abdominal pain and no nausea or vomiting; hence, she was admitted to our hospital for further diagnosis and treatment. The patient’s body temperature was 36.8°C, pulse rate was 87 beats/min, respiration rate was 18 breaths/min, and blood pressure was 125/76 mmHg. The patient was conscious and had a thin appearance, and physical examination indicated that the abdomen was soft, and without tenderness and rebound pain. Routine blood examination showed that hemoglobin was 87 g/L and hematocrit was 28.3%. Abdominal computed tomography revealed the right lower abdominal small intestine protruding into the femoral triangle through the obturator; the upstream intestine was distended and expanded, and multiple fluid levels were observed in the intestinal lumen. Multiple lymph nodes were noticed in the abdomen and retroperitoneum; the large ones had a diameter of ~1 cm. Uneven thickening and enhancement of the local ascending colon were noted.Laparoscopic exploratory laparotomy and obturator hernia repair were performed. A 4 × 4 cm thick hard tumor was observed in the ascending colon near the liver curve, involving serosa, and the ascending colon was partially blocked. A total of 80 cm away from ileocecum, the intestine entered from the right obturator hernia, causing obstruction and expansion of the proximal intestinal canal, congestion, and edema of the intestinal wall. We aspirated the ascites, loosened the adhesion around the obturator hernia ring, and returned the small intestine back into the abdominal cavity. It was observed that the intestinal wall of the hernia section was ~4 cm, necrotic, and broken, and the intestinal contents were flowing out, the intestinal defect was immediately closed, the abdominal cavity was flushed with normal saline, and the peritoneum at the obturator was intermittently sutured and repaired. A median abdominal incision of ~12 cm was used to cut each layer of the abdominal wall, enter the abdomen, protect the incision, lift out the intestine at the breach, place the aspirator in the intestinal lumen, aspirate large amount of intestinal contents, and temporarily close the breach. The abdominal and pelvic cavities were rinsed again with plenty of normal saline. With the consent of the patient’s family, a double-cavity ileostomy was successfully performed.To date, the concurrent occurrence of closed hernia and colon cancer has not been reported. This case is the only confirmed case of this type, although the tumor was not removed. We discussed the causes of intestinal obstruction, the surgical methods used, and the use of surgical mesh patches.The patient was admitted due to intestinal obstruction. Some experts believe intestinal obstruction is caused by intestinal tumors and that obturator hernias are related to them, whereas others propose the underlying cause is a combination of an obturator hernia and colon cancer. We believe the latter is correct because first, our patient was an older woman who was prone to having an obturator hernia due to repeated vaginal delivery and low weight; second, she occasionally complained of thigh pain. During the surgery, it was confirmed that the small intestine had entered the femoral triangle, causing thigh pain; and third, a colonic tumor was discovered during the operation, which caused partial blockage of the ascending colon, the small intestine had extended into the femoral triangle, causing partial necrosis of the small intestine. Regarding the surgical method, some surgeons believe that obturator hernia repair should be performed simultaneously with colon tumor resection, whereas others support performing only the obturator hernia repair. During the operation, the family members were informed of this fact; they agreed, and only obturator hernia repair was conducted, which we preferred. The reasons are as follows: the patient was 86 years old, thin, had a hemoglobin of 87 g/L, and could not have tolerated simultaneous resection. In addition, she had a severe chest infection, abdominal infection, intestinal wall edema, and expansion caused by intestinal obstruction, and an ascending colon tumor. If the obturator hernia repair was performed at the same time as the colon tumor resection, there might have been a risk of leakage of the intestinal anastomosis and aggravation of the ascending colon obstruction. Therefore, major surgery for concurrent removal of the ascending colon tumor was not considered.et al. [There are conflicting opinions about the use of mesh patches. We prefer not to use mesh patches. Laparoscopic mesh placement is the most appropriate treatment, although only under the right patient conditions . Hosoi eet al.  believe et al. . Our patThis is the first reported case of concurrent occlusive hernia and colon cancer. The main symptoms were intestinal obstruction, including nausea, vomiting, and constipation. The decision whether the tumor should be removed simultaneously with herniorrhaphy, and/or using the mesh patch during herniorrhaphy depends on the comprehensive situation of the patient.


Type B lactic acidosis is an uncommon medical emergency in which acid production overwhelms hepatic clearance. This specific etiology of lactic acidosis occurs without organ hypoperfusion and has been most commonly described in patients with hematologic malignancies but also in patients with solid tumors. The mechanism by which cancer cells switch their glucose metabolism toward increasingly anaerobic glycolytic phenotypes has been described as the “Warburg effect.” Without treating the underlying malignancy, the prognosis for patients diagnosed with malignancy-related type B lactic acidosis is extremely poor. Here, we present a case of a 66-year-old male who was diagnosed with type B lactic acidosis secondary to mantle cell lymphoma. Bicarbonate drip was started to correct the lactic acidosis. The patient was also immediately treated with rituximab chemotherapy combined with rasburicase to avoid the hyperuricemia associated with tumor lysis syndrome. He responded to the early treatment and was discharged with normal renal function. Type B lactic acidosis secondary to hematologic malignancy is important to recognize. In order to successfully treat this syndrome, early diagnosis and simultaneous treatment of the imbalance of lactic acid levels and the underlying malignancy are necessary. Lactic acidosis is a medical emergency that can result in severe hemodynamic consequences. The most common etiology is type A lactic acidosis, which often reflects tissue hypoxia due to hypoperfusion –3. A minα  is a rare and aggressive form of B-cell non-Hodgkin's lymphoma. It is rarely associated with type B lactic acidosis via the Warburg effect . Here, wA 66-year-old male with history of diabetes mellitus, cardioembolic stroke, and patent foramen ovale was diagnosed with MCL two weeks prior to presentation. Diagnosis of MCL was confirmed by immunohistochemical studies of neoplastic cells from right axillary lymph nodes that were positive for CD20, PAX5, CD5, cyclin D1, CD43, and BCL2 tumor markers associated with MCL . He had Clostridium difficile toxin specimen of the stool. A computed tomography scan of the abdomen and pelvis was done revealing extensive retroperitoneal and intra-abdominal lymphadenopathy with splenomegaly. There was no evidence of bowel ischemia or hydronephrosis that could be contributing to the lactic acidosis. After multiple consultations, a diagnosis of type B lactic acidosis secondary to MCL was made. He was given a fluid challenge as well as intravenous rituximab with rasburicase to prevent acute urate nephropathy due to his chronic renal insufficiency.On the second day of admission, the patient's lactic acid level increased to 7.0 mMol/L, and he was noted to have abdominal pain with a positive By the fourth day of admission, the patient's clinical condition started to improve, and he was transitioned out of the intensive care unit. His bicarbonate levels improved, so the bicarbonate drip was terminated, and he was started on normal saline. He continued to respond to therapy with normalization of creatinine (1.13 mg/dL), BUN (19 mg/dL), and serum lactic acid levels. Supportive care was continued for ongoing volume overload and acute kidney injury, and he was discharged two weeks after the diagnosis of type B lactic acidosis.The “Warburg effect,” which was initially described in 1923, provides the most complete explanation of the hypothesized mechanism behind type B lactic acidosis in malignancies and is an increasingly recognized hallmark of cancer cells . Cancer Type B lactic acidosis has been described in conjunction with various hematologic malignancies and occasionally in cases involving solid tumors –7, 26. PHematological malignancies, which inherently impact both the circulatory and lymphatic systems, present serious challenges to physicians attempting to treat the systemic consequences of the disease. Type B lactic acidosis has been reported with hematologic malignancies such as acute and chronic leukemias, histiocytosis, and Hodgkin's lymphoma. A literature review regarding type B lactic acidosis secondary to malignancy demonstrated that type B lactic acidosis served as a poor prognostic marker for patients and that most cases were fatal despite intense intervention . PreviouThere are very few reported cases of MCL associated with type B lactic acidosis , 33–36. When treating patients with hematological malignancies that present with idiopathic degeneration of respiratory status, type B lactic acidosis should be considered as a possible diagnosis. Metformin-associated lactic acidosis is a much more common form of significantly elevated lactic acidosis which often responds to aggressive treatment with intravenous fluids, bicarbonate, and renal replacement therapy when indicated –41. In o


Circumscribed Choroidal hemangioma (CCH) is a kind of hamartoma that is caused by congenital vascular malformation. And, polypoidal choroidal vasculopathy (PCV) is an exudative maculopathy. There is no literature indicating that there is a correlation between the occurrences of CCH and PCV.A 66-year-old male presented with decreased vision of his left eye for 4 years. Fundus photograph showed that the branches of blood vessels at the supratemporal retina were occluded in white lines, an orange lesion could be seen in the subnasal retina and mottled, yellowish white lesions were accompanied by punctate hard exudation in the macular in the left eye. The Fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and Spectral domain optical coherence tomography (OCT) were done. There was a diagnosis of CCH, PCV and branch retinal vein occlusion accompanied with retinoschisis of the left eye.This article reports on a case of an elderly male Chinese patient with CCH and PCV accompanied by branch retinal vein occlusion with retinoschisis in the left eye. The common lesions are choroidal vascular abnormalities. Whether hypertension is related to CCH, PCV and branch retinal vein occlusion remains to be further studied. Choroidal hemangioma is a kind of hamartoma that is caused by congenital vascular malformation, which can be divided into diffuse and circumscribed types . PatientPolypoidal choroidal vasculopathy (PCV) is an exudative maculopathy characterised by multiple recurrent serosanguineous retinal pigment epithelial detachments . YannuzzThis article reports on a case of an elderly male Chinese patient with CCH and PCV accompanied by branch retinal vein occlusion with retinoschisis in the left eye.A 66-year-old male presented with decreased vision accompanied by metamorphopsia of his left eye for 4 years. During the 4 years, he didn’t see a doctor and had not received any treatment. He had no systemic diseases, such as diabetes or heart disease. His blood pressure was 140/110 mmHg. The best corrected vision  was 0.8 in his right eye and finger counting in his left eye. The intraocular pressure was within normal limits in both eyes. There was light opacity in the bilateral lens. A fundus examination  showed that the branches of blood vessels at the supratemporal retina were occluded in white lines, an orange lesion could be seen in the subnasal retina and mottled, yellowish white lesions were accompanied by punctate hard exudation in the macular in the left eye   revealed a double-layer sign and sharp-peaked pigment epithelial detachment in the macular Fig. F, as welThe CCH is a benign vascular tumour that is not accompanied by facial or systemic diseases . The maiWhat makes the patient in this article special is that he had CCH, but also had PCV and branch retinal vein occlusion simultaneously. It is unknown whether there is a relationship between the occurrence of CCH and PCV. Polypoidal choroidal vasculopathy was similar to neovascular, age-related macular degeneration in morphological characteristics,  which waFurthermore, the patient suffered from retinoschisis at the lesion of branch retinal vein occlusion. The mechanism may be that the longer course of branch retinal vein occlusion leads to retinal capillary blood supply disorder, which causes retinal cell apoptosis and inner retinal cystic degeneration. Then, the cortex covering the vitreous body shrinks, causing traction of the inner retina, and finally forming retinoschisis.The therapy of simple CCH contains laser photocoagulation and PDT. This patient refused to do laser photocoagulation because of the possible risks. And we did not have the PDT treatment at present. The therapy of our case should contain intravitreal injection of anti-VEGF drugs or PDT for PCV and laser photocoagulation for BRVO or CCH. However, the patient only received intravitreal injection of anti-VEGF drugs twice and there was not much effect. He gave up the treatment at last.In conclusion, this article is the first report of a rare case of simultaneous CCH, PCV and branch retinal vein occlusion, as far as we know. The common lesions are choroidal vascular abnormalities. Whether hypertension is related to CCH, PCV and branch retinal vein occlusion remains to be further studied.


About half of osteosarcomas occur near the knee joint, but other sites such as the humerus, upper femur, fibula, spine, and ilium can also occur. However, rib osteosarcoma is rarely reported. Here, we report the case of a 17‐year‐old female who was found to have a left dorsal mass on physical examination. Computed tomography (CT) revealed bone destruction in the seventh rib, leading to surgery for mass excision. Pathological results suggested chondroblastic osteosarcoma. After surgery, the patient was treated with chemotherapy and is doing well. Osteosarcoma of the ribs is very rare. Here, we report a case of primary osteosarcoma of the ribs in an adolescent girl. The patient underwent complete resection of the rib mass, and the mass was histopathologically diagnosed as chondroblastic osteosarcoma. After surgery, the patient received adjuvant chemotherapy and has no evidence of recurrence. It is mainly manifested as the formation of immature osteoid cells by tumor cells. Osteosarcoma is the most common primary bone tumor in children and adolescents, with a tendency to occur in the metaphysis of the long shaft. Osteosarcoma occurring at the rib end is very rare, and here, we report a case of primary rib osteosarcoma treated in an adolescent girl in order to provide some diagnostic aid to clinicians.2We admitted a 17‐year‐old adolescent girl. She has a 1‐month history of swelling and pain in her left chest and back. During a school‐organized physical examination 4 days ago, a lump was found on her left back. The patient has no symptoms such as fever, cough, dyspnea, and weight loss. We examined the patient and found that a lump could be felt at the level of approximately the seventh rib on the left chest and back, with an area of approximately 2.0 × 3.0 cm. The lump feels tough and lacks mobility, causing pain when pressed on this area. Complete blood cell count and serum biochemistry are within the normal range. Chest x‐ray showed rib destruction and surrounding mass. The results of chest computed tomography (CT) showed that the seventh rib on the left side of the patient was partially damaged, with a soft tissue mass surrounding it, and no pleural effusion was found . According to our domestic treatment plan, surgery can be performed before chemotherapy. For stage IIB and III patients, neoadjuvant chemotherapy followed by surgery, and then chemotherapy. Preoperative neoadjuvant chemotherapy is very important for patients with limb salvage, but this patient's osteosarcoma is located in the ribs, and there is no need to reconstruct the bone structure. With or without preoperative chemotherapy, complete resection can be achieved without affecting the function. For patients with stage IIA rib osteosarcoma, early resection of the tumor also has certain advantages. For example, patients have good immunity and quick recovery after surgery. In addition, it can avoid some adverse consequences caused by puncture and obtain reliable pathological results at an early stage.Xiaofeng Hu: Study concept or design; data collection. Tianyi Bao: Data analysis or interpretation; writing the paper. Chao Yan: data collection. Yongliang Zhu: Data analysis or interpretation. Xiaofei Zheng: Study concept or design; writing the paper.The authors declare that they have no conflicts of interest.Appropriate written informed consent was obtained from the patient for the publication of this case report and accompanying images. It was approved by the Clinical Research Ethics Committee of Jinling Hospital of Nanjing Medical University and was implemented.


We present an intriguing case of an incarcerated femoral hernia containing an inflamed appendix inside the sac, also known as a De Garengeot hernia. This type of hernia is a rare occurrence that was first described in 1731 by the French surgeon René-Jacque Croissant de Garengeot. A 64-year-old woman presented to the emergency department with a painful mass in the right groin region. Following a computed tomography (CT) scan of the abdomen and pelvis to evaluate the mass, the diagnosis of a femoral hernia containing a strangulated appendix was established. Subsequently, a hybrid surgical approach was utilized, consisting of an open hernia repair and a laparoscopic appendectomy. A hernia is defined as the protrusion of any intraperitoneal organ or part of it through a weak area or hole in the abdominal wall. The classification of a hernia is based on its location and the content of the hernia sac. The most commonly occurring types of hernias include inguinal hernias, femoral hernias, incisional hernias, umbilical hernias, and epigastric hernias .InguinaWe report the case of a 64-year-old woman who presented to the emergency room with sudden-onset pain in her right groin following a weightlifting incident. She did not report any symptoms of bowel obstruction or any significant medical history.Physical examination revealed a tender, non-mobile, and painful mass in the right groin. Blood tests showed, respectively, normal levels of C-reactive protein 0.1 mg/dL) and white blood cell count (7.8K/μL). A CT scan of the abdomen and pelvis was performed, revealing a right femoral hernia containing a strangulated appendix  scan of the abdomen and pelvis can be a valuable diagnostic tool to aid in the preoperative diagnosis of a De Garengeot hernia .An immediate and accurate diagnosis is crucial in determining the appropriate surgical plan. A majority of femoral hernias present as an emergency condition due to the narrow neck of the femoral ring. Due to the rarity of this type of hernia, no specific surgical technique is used. In most cases, the inguinal approach is preferred, which allows for both hernia repair and appendectomy. In cases where laparotomy is selected, it is due to the quality or accessibility of the base of the appendix and the need for exploration of the abdominal cavity. For cases requiring laparotomies, a McBurney incision or midline incision is typically chosen for better access .In the inguinal approach, the McEvedy technique  appears to lead to fewer laparotomies compared to the Lockwood  and Lotheissen's approaches . In cases like ours, there is a choice to combine laparoscopic appendectomy and open hernia repair. Laparoscopy provides the option to explore the abdomen and perform a laparoscopic appendectomy. In some cases, the laparoscopic approach was used for both appendectomy and hernia repair. Transabdominal preperitoneal repair (TAPP) and total extraperitoneal repair (TEP) have also been used in combination with laparoscopic appendectomy in the case of Garengereot hernias .Regarding hernia repair, sutures were predominantly used. However, in selected cases where there were no indications of severe inflammation of the appendix, a mesh was employed.In this case, the patient underwent a successful open hernia repair and laparoscopic appendectomy. By means of laparoscopy, we had the opportunity to assess the abdominal cavity and perform a laparoscopic appendectomy. The preoperative diagnosis of a De Garengeot hernia was essential in selecting the appropriate surgical approach for our patient.This case underscores the significance of an expeditious and comprehensive assessment of a femoral hernia, as it may harbor diverse contents that have the potential to result in grave complications. The effective management of our patient's De Garengeot hernia necessitated a prompt and precise diagnosis, followed by appropriate surgical intervention. Therefore, it is imperative to incorporate the possibility of a De Garengeot hernia in the differential diagnosis of all patients who present with a groin mass.


Leiomyosarcoma of visceral organs is uncommon, and pancreatic primary occurrence is even rarer. In terms of curative treatment, patients are generally managed with surgery alone, without significant data on the role or efficacy of adjuvant chemotherapy.This manuscript presents a case of a 22‐year‐old female with advanced primary leiomyosarcoma of the pancreas, treated with radical surgery and adjuvant radiation therapy.With a low‐survival rate, consideration of radiation therapy in some advanced and unresectable cases could be potentially beneficial. Primary leiomyosarcoma of the pancreas (PLMS) is a rare event, accounting for two of every 5075 cases of leiomyosarcomas,Due to the rarity of the disease and the lack of evidence for best practices, its management is controversial. A comprehensive search revealed reports of the disease that mainly focused on clinicopathologic and imaging features, and revealed that many patients were managed with surgery alone. This case report presents a young female patient with PLMS, invading the peripancreatic tissue, who was treated with radical surgery and adjuvant radiation therapy,2A 22‐year‐old female presented to a gastroenterologist in Mashhad, Iran in March 2020, with the chief complaint of abdominal pain lasting for more than 1 year. The pain had progressively worsened and she had developed icterus a week prior to her presentation. She did not have any loss of weight, and no other comorbidities.The initial abdominal ultrasound revealed dilation of bile ducts. Computed tomography (CT) scan illustrated a hypodense mass in the head of pancreas, suspected to be a focal pancreatitis or a pancreatic mass Figure . MagnetiGross pathological examination demonstrated a firm, creamy, infiltrating tumor mass, measuring 4.5 × 4 × 4 cm, located in the pancreatic head, invading the peripancreatic tissue and an adjacent lymph node. The tumor had focally invaded the duodenal wall, but did not seem to be originating from it. Microscopic and immunohistochemistry studies identified the tumor as leiomyosarcoma of the pancreas Figure .By May 2020, 1 month postsurgery, she was completely active with an Eastern Cooperative Oncology Group (ECOG) Performance Status score of 0, and free of symptoms. On physical examination neither organomegaly nor tenderness of the abdomen were detected. After presenting the case to a multidisciplinary care team, and discussing the benefits and drawbacks of all available adjuvant options, she was then further treated with adjuvant external beam radiotherapy at a dose of 5040 cGy in 28 fractions by intensity modulated radiotherapy technique on the basis of invasion to neighboring organs.After 20 months of follow‐up, she has been disease free as evidenced by chest and abdominopelvic scans obtained every 3 months. She is completely active with an ECOG Performance Status of one. She only complains of abdominal discomfort, weight loss, digestive problems, and chronic fatigue.3Pancreatic leiomyosarcoma is a rare mesenchymal tumor accounting for less than 0.1% of all malignant pancreatic (non‐islet) cancers. This malignancy has a uniquely aggressive behavior that affects both adjacent and distant organs. The rarity of this disease and the lack of research and available data on most effective treatments, particularly in young patients, demonstrates the need for this case report and others like it.First described by Ross in 1951, thereafter, cases of pancreatic leiomyosarcoma have primarily been addressed through case reports and managed by radical resection; however, a multidisciplinary team decided to treat our case with adjuvant radiotherapy due to focal invasion of neighboring organ. A recent review of clinicopathological features of 88 patients with primary leiomyosarcoma of the pancreas showed that most cases occurred during the fifth decade of life with the youngest being 14 years old.While patient age, tumor size, vascular invasion, and mitotic count have generally been proposed as important prognostic factors for leiomyosarcoma, a systematic review demonstrated slightly different results in leiomyosarcomas of pancreas: nonradical resection was an independent adverse prognostic factor, invasion to adjacent organs/vessels was a potential adverse factor, while size and age showed no significant effect.However, the most important prognostic factor agreed upon in the literature is radical surgery.However, some cases with local invasion to adjacent organs with durable disease‐free survival have also been reported. For example, a case of PLMSP in the head of the pancreas with invasion into the ampullary region and common bile duct, was disease free 19 months postsurgery, without any adjuvant treatment.Data on the use of adjuvant treatment in this disease is extremely lacking. To the best of our knowledge, only 3 reported cases underwent adjuvant treatment Table . A 40‐yeThe national comprehensive cancer network guidelines recommend consideration of pelvic EBRT in completely resected stage II‐III  uterine leiomyosarcoma.The effectiveness of RT is further demonstrated in a report of an unresectable, nonmetastatic PLMSP patient who refused chemotherapy and underwent palliative radiotherapy instead. He received 54Gy over 30 fractions and was clinically well for 18 months.4The primary leiomyosarcoma of the pancreas is an extremely rare entity and data about its diagnostic consideration and management is grossly insufficient. However, due to high mortality rates, adjuvant treatments following a Whipple procedure is crucial. While requiring longer follow‐ups, there is cautious optimism about the potential benefits of radiation therapy in the management of this rare malignancy.Ali Taghizadeh Kermani: conceptualization ; supervision (lead); resources ; writing – review and editing . Alireza Khooei: Data curation ; resources ; writing – original draft (supporting); writing – review and editing (supporting). Mohsen Aliakbarian: data curation ; resources ; supervision (supporting); writing – review and editing (supporting). Soudeh Arastouei: data curation ; writing – original draft (lead); conceptualization (lead); Writing – review and editing .The authors listed are the sole authors of this manuscript. The authors received no specific funding for this work.The author declares there is no potential conflict of interest.A written informed consent was obtained from the patient for this manuscript.