Neurofibromatosis type 1 (NF1) is an autosomal dominant condition with a high rate of new mutation and variable expression. Diffuse neurofibroma of the epidermis invading deeper organs is rare.We report a case of diffuse subcutaneous neurofibroma in the thoracoabdominal wall which had invaded the diaphragm and caused diaphragmatic eventration.We describe a patient with diffuse neurofibroma of the chest and abdomen who was admitted to the hospital due to sudden abdominal pain and a possible diaphragmatic hernia. We performed thoracotomy and found that the neurofibroma had invaded the diaphragm and caused diaphragmatic eventration.This occurrence has not been reported, and it shows that although neurofibromatosis is a benign disease, it still has the biological behavior of a malignant tumor and may cause a serious impact on and damage to other organs. Neurofibromatosis type 1 (NF1) is an autosomal dominant condition with a birth incidence of approximately 1:3000 . NF1 wasA 24-year-old male presented with diffuse skin lesions on the chest and abdominal walls from the age of 8, accompanied by pigmentation and epidermal nodular hyperplasia. Because of sudden spontaneous swelling and pain in the left upper abdomen that could not be relieved by rest or changing body position, he went to the emergency department of our hospital. The main vital signs of the patient were body temperature of 37.8℃, blood pressure of 140/93 mmHg, respiration of 24 beats/min, heart rate of 115 beats/min, and oxygen saturation of 96%. The emergency chest and abdominal enhanced CT scan showed the following: possible left diaphragmatic hernia; partially inflated left lung; massive left abdominal mass with hemorrhage, with the left 9th/11th ribs partially absorbed; left chest and back subcutaneous soft tissue thickened with an abnormal density; and bleeding nodules (shadow) on the chest and back skin , and abdominal muscle tension.Based on the patient's condition and CT findings, the possibility of a diaphragmatic hernia caused by spontaneous diaphragmatic rupture was not ruled out. After communicating with the patient's family members, we decided to perform thoracotomy. We selected the left 7th intercostal incision site, from the skin to the subcutaneous tissue there were a lot of neurofibroma tissue, brittle tissue and easy to bleed. The tumor covered a wide area, including the lateral chest wall, the back, and the whole abdomen. Because large areas of tumor tissue cannot be completely removed, only the thorax and abdominal problems can be dealt with. The left pleura and diaphragm were found to be invaded by tumor during the operation. The left diaphragm appears dark green with thinning tissue and reduced elasticity. The left diaphragm is significantly elevated, but not ruptured. Compression atelectasis was observed in the left lung. A small amount of bloody effusion was found in the abdominal cavity, and a portion of the bowel was embedded in the very large, twisted subcutaneous neurofibroma without necrosis. The bloody effusion was considered to be bloody exudation caused by intestinal cantun, and there is no obvious active bleeding in abdominal cavity. We found no obvious bleeding and necrosis in the abdominal cavity, and then a drainage tube was been placed. Diaphragmatic folding was performed. A portion of subcutaneous tissue and various diaphragmatic muscle tissues were removed during the operation and sent for rapid pathology. The diagnosis was neurofibroma Fig. . The pat"Neurofibromatoses" are a set of distinct genetic disorders that have in common the occurrence of tumors of the nerve sheath. They include NF1 and NF2 neurofibromas and schwannomas. NF1 affects multiple systems of the body. The major NF1-associated tumor is neurofibroma. In addition, clinical manifestations include bone dysplasia, learning disabilities, and an increased risk of malignancy . NeurofiDiffuse neurofibroma frequently grows as a plaque-like or infiltrative lesion involving the skin and subcutaneous tissues. Prominent internal vascularity is common. The mean patient age is 35.1 years . DiffuseThis case shows that although neurofibromatosis is a benign disease, it still has the biological behavior of a malignant tumor and may cause a serious impact on and damage to other organs. The surgical treatment of diffuse neurofibromatosis is limited, and early intervention  may be a breakthrough in the future.


The average and maximal standardized uptake values (SUVs) were 1.92 and 2.29, respectively. The tumor-to-normal-tissue (T/N) ratio was 0.97. The first evidence of clinical deterioration was noticed on day 1147. On day 1155, 3,4-dihydroxy-6-[18F]-fluoro-l-phenylalanine (18F-FDOPA)-PET was performed. High uptake of 18F-FDOPA was observed in the intracranial lesion. The mean and maximal SUVs of the tumor were 1.59 and 2.29, respectively. The T/N ratio was 2.22. The patient was euthanized on day 1155 and histopathologic evaluations confirmed glioma (astrocytoma). This case shows that chemotherapy with hydroxyurea plus imatinib may be considered in the treatment of canine glioma. Furthermore, this is the first case describing the application of 18F-FDG and 18F-FDOPA in a dog with glioma.An 8-year-old neutered male Yorkshire Terrier dog presented with head pressing, vestibular ataxia, neck tenderness, and no oculocephalic reflex. A demarcated lesion in the pons was identified on MRI. The patient was tentatively diagnosed with a glioma and was treated with hydroxyurea plus imatinib and prednisolone. After 30 days of therapeutic treatment, the patient showed a clear improvement in neurological signs, which lasted for 1117 days. On day 569 after the initiation of treatment,  An 8-year-old neutered male Yorkshire Terrier dog presented with a history of head pressing, and an episode of vestibular ataxia was observed 2 days prior to presentation. On physical examination, the patient weighed 5.3 kg, had a pulse rate of 116 beats per minute, a respiratory rate of 48 breaths per minute, and a rectal temperature of 38.9 °C. On neurological examination, the patient exhibited neck pain, and the oculocephalic reflex was not elicited. Biochemical analysis did not show any remarkable abnormalities except for mildly increased alkaline phosphatase activity. Based on the clinical signs and findings on neurological examination, the lesion was neuroanatomically localized to the brainstem.TM, GE Healthcare (Shanghai), Co., Ltd, China], post-contrast images were acquired, and ring enhancement was noted , a glioma was strongly suspected.MRI of the brain was performed using a 0.3-Tesla unit . General anesthesia was induced with intravenous administration of propofol  and maintained by inhalation of 2.0–2.5% isoflurane  in 100% oxygen in a circle rebreathing circuit. T1-weighted (pre- and post-contrast), T2-weighted, and fluid-attenuated inversion recovery (FLAIR) images were obtained using transverse, sagittal, and dorsal planes. The lesion was identified in the pons, and peritumoral edema was found around the lesion. Hyperintensity in T2-weighted  and FLAIas noted . CerebroFor metastatic evaluation, the patient underwent radiography and ultrasonography on the day after the MRI scan, and no metastatic lesion was observed.®, Yuhan, Seoul, South Korea) and lomustine . Nine days after the commencement of chemotherapy, the patient experienced severe vomiting and hyporexia. Myelosuppression due to lomustine was not observed. The neurological signs did not improve; therefore, the chemotherapy was modified as follows: the lomustine was stopped, and a new regimen consisting of hydroxyurea  plus imatinib  and prednisolone  was initiated. At 30 days after the initial chemotherapy, the slight leaning to the left improved, and the oculocephalic reflex was elicited. Therefore, a second MRI was performed to evaluate the therapeutic effectiveness through comparative analysis of tumor size. Although peritumoral edema was increased compared with that on the previous MRI finding, a decreased contrast-enhancing tumor size was observed on the second MRI  and lymphopenia , hydroxyurea was tapered to 30 mg/kg BW. At 213 days after the commencement of chemotherapy, the patient still showed a partial improvement in neurological signs, and there were no adverse effects of chemotherapy. A third MRI was performed to evaluate the therapeutic effectiveness on day 213. The third MRI showed decreased peritumoral edema compared with the findings of the previous scans -positron emission tomography (PET) scan of the whole body, including the head, was performed immediately after the fourth MRI scan to determine the malignancy of the tumor and whether metastasis had occurred. 18F-FDG (5.92 MBq/kg BW) was administered intravenously into the right saphenous vein, followed by 5 mL of 0.9% normal saline for flushing of residual 18F-FDG. Low-dose CT images were acquired before PET scanning. Twenty-minute PET scans were obtained one hour after 18F-FDG injection. PET image analysis was performed using the OsiriX MD v10.0 . The regions of interest (ROIs) were drawn manually on the PET/CT fusion images. The metabolic activity of the ROIs was converted to a standardized uptake value (SUV) as follows: SUV = average tissue concentration of 18F-FDG (MBq/mL)/injected dose (MBq) per body weight (g). From a visual evaluation of the PET image, 18F-FDG uptake of the tumor was not remarkable compared with the surrounding region of the tumor  and 1 mg/kg BW (twice daily), respectively. Despite the increased doses of hydroxyurea and prednisolone, the patient did not show signs of recovery; therefore, a fifth MRI and 3,4-dihydroxy-6-[18F]-fluoro-l-phenylalanine (18F-FDOPA) PET were performed to identify the tumor size at day 1155 after the initial chemotherapy. The fifth MRI was performed with a 1.5-Tesla unit MRI system . The tumor appeared much larger on the fifth MRI scan than on the previous scans  and interval time (10 min) from injection to scanning. On 18F-FDOPA PET/CT imaging, a high uptake of 18F-FDOPA was noted in the lesion, and protrusion of the tumor mass resulting from bone lysis was also identified  and platelet-derived growth factor receptor (PDGFR)-β antibodies were used for immunohistochemistry (IHC) analysis. Anti-LAT1 and anti-PDGFR-β antibodies were purchased from Abcam . The VECTASTAIN elite ABC-HRP kit was obtained from Vector Laboratories . LAT1 IHC staining revealed that the tumor mass was strongly positive in the cytoplasm, plasma membranes, and vascular endothelium surrounding the local extensive area of necrosis . In addi18F-FDG PET has been used to distinguish benign from malignant tumors  has been reported in the dog species  is much better than 18F-FDG PET (61%) for the visualization of human gliomas , and the T/N ratios of canine low-grade glioma in 18F-FDG PET and 18F-FDOPA PET were 0.97 and 2.22, respectively. Although both the SUVmax values of canine low-grade glioma were lower than those of humans, the T/N ratio, which is an objectively quantified value, was similar in humans and dogs.In human oncology, The oculocephalic reflex (physiologic nystagmus) requires the vestibular system and its connected components to be normal. However, when bilateral defects in the vestibular system and its related connections occur, physiologic nystagmus is not elicited. Therefore, the recovery of physiologic nystagmus, which was not initially observed, was due to the improvement of the lesion in the brainstem after chemotherapy with prednisolone was initiated.Several treatment methods, including surgery, radiotherapy, symptomatic management, chemotherapy, and combined therapies, have been used in canine brain tumors  (Kanai et al. 18F-FDG and 18F-FDOPA PET results in a dog with glioma. In the present case, because the tumor was not detected by 18F-FDG, but by 18F-FDOPA PET, the latter can be used for a better identification of low-grade gliomas. This data may provide valuable diagnostic information for understanding canine gliomas in veterinary medicine, and further studies are needed to establish diagnostic and staging criteria for canine gliomas. Furthermore, there have been no previous reports of canine gliomas treated only with a chemotherapy combination of hydroxyurea and imatinib. Our findings suggest that chemotherapy with hydroxyurea and imatinib may be considered as an alternative treatment for canine gliomas when surgery and radiotherapy are limited for various reasons.To the best of our knowledge, this is the first reported case of


Beckwith–Wiedemann syndrome (BWS) is a rare congenital condition characterized by complex overgrowth of different body parts. Children with Beckwith–Wiedemann syndrome, particularly those with hemihypertrophy, experience an increased risk of developing benign and malignant tumors. This case report presents an 18-month-old girl with features suggestive of Beckwith–Wiedemann syndrome who developed pubic hair, high levels of testosterone, and DHEAS with normal cortisol and progesterone levels. Computed tomography revealed a left adrenal mass. Histopathological examination of the resected mass showed an adrenocortical tumor. Her postoperative evaluation showed normal testosterone and DHEAS levels. Early diagnosis and detection of intra-abdominal neoplasms in infants with Beckwith–Wiedemann syndrome are essential to avoid serious clinical complications. Beckwith–Wiedemann syndrome (BWS) is also termed EMG  syndrome. Historically, the first clinical description was in 1960 by John Bruce Beckwith and Hans-Rudolf Wiedemann .BWS has a highly variable manifestation; abnormal growth is the most common feature which can result in macrosomia, hemihypertrophy, or enlarged tongue. Other presentations include abdominal wall defects, visceromegaly, ear lobe creases, cleft palate, renal alterations, refractory hyperinsulinemia, and polydactyly. Neurologic complications are rare . BWS patth percentile). There was no family history of genetic disorders.An 18-month-old female child was referred with a 5-month history of excessive pubic hair (pubarche). She was born at full term by normal spontaneous vaginal delivery, following an uncomplicated pregnancy with no polyhydramnios. No congenital malformations were noticed at birth like exomphalos and macroglossia. She was noted to have left-sided hemihypertrophy involving the trunk and limbs without any management. She had neither hypoglycemic attacks nor omphalocele. Her birth weight was 4000 g (50th percentile), the length 89 cm (50th percentile), temperature 37°C, oxygen saturation 98%, and arterial blood pressure 90/55 mm Hg. She looked well with whole left-sided hemihypertrophy, umbilical hernia, ear lobe creases, and pubic hair. She had no axillary hair growth, acne, breast enlargement, or vaginal bleeding. No rapid weight or height gain was noticed. Her Tanner staging was A1, P2, and B1. Laboratory investigation reports were as in On examination, her body weight was 15 kg  are rare in children , and their incidence is increased by 40-fold in BWS patients. More than 90% of them are associated with the active secretion of adrenocortical hormones including androgens, estrogens, cortisol, and aldosterone. Most of these tumors have been suggested as carcinomas, and pure virilizing adenomas are rare . In the Our case was not diagnosed as a case of BWS till 18 months of age and regular screening was not carried out. The child presented with only excessive pubic hair (pubarche), and this was the chief complaint that leads to the diagnosis of BWS.Histopathologically, the tumor has a different pattern of growth that makes distinguishing between benign and malignant neoplasms difficult. Wieneke index, which requires histopathological findings, is employed to distinguish benign from malignant tumors. This classification is the most accurate score in children. Weineke score consists of the tumour size weight, size, tumor extension , mitotic index, confluent necrosis, and atypical mitosis. The tumor is rendered as benign when the score is two or less [3-1].In our case, considering the weight and size of the tumor, coupled with the microscopy, elevated hormonal levels, Wieneke score, and the clinical symptoms, a diagnosis of ACA was made. The tumor was completely removed without cortisol replacement.A functional adrenocortical tumor has rarely been documented in a child, but it is increased in the presence of BWS. Early abdominal ultrasound is recommended in infants with BWS due to high suspicion for neoplasms. Early diagnosis and treatment are essential to avoid serious clinical complications.


Paroxysmal positional vertigo is a frequent cause for consultation. When approaching these patients, we try to differentiate central from peripheral causes, but sometimes we find manifestations that generate diagnostic doubts. In this review, we address atypical paroxysmal positional vertigo, reviewing the literature on the subject and giving a provisional definition of atypical positional vertigo as well as outlining its causes and pathophysiological mechanisms. Benign paroxysmal positional vertigo is the most frequent cause of vertigo . As its Based on what patients report, the duration of symptoms lasts seconds; however, many overestimate the duration of the vertiginous sensation. The trigger effect of positional changes is a key issue to be addressed . A greatTherefore, we may define PPV as a condition characterized by sudden-onset and -end vertigo episodes, triggered by changes in the head’s position with regard to gravity.If, after questioning, the diagnosis is PPV, there is an attempt to find the cause of these symptoms by performing a physical examination and, in certain cases, supplementary tests. The causes may be divided in two main groups: Peripheral, as in the case of Benign Paroxysmal Positional Vertigo (BPPV), in its typical and atypical forms , and posPPV associated to neurological disorder signs and symptoms;Nystagmus without vertigo in positional maneuvers;Atypical direction nystagmus, especially downbeating nystagmus;Nystagmus that changes direction during the positional test;Poor response to repositioning maneuvers;Recurrence on more than three occasions, confirmed by positional tests.In 1999, Büttner et al. published criteria to help differentiate between BPPV and CPPV . Soto-VaThese elements are usually useful to differentiate the aforementioned clinical pictures when they are typical . In praBelow, we provide a brief review of some of the PPV causes that, due to their characteristics, may result in diagnostic doubts. We leave aside the description of typical BPPV pictures (see ), but prIn most patients, BPPV presents with a typical description and physical examination, with no diagnostic difficulties. However, in some cases, there are hard-to-explain findings, which may result in diagnostic error.If we were to use a ladder to plot the degree of atypicality, we may find what is shown in In recent years, many cases and series of cases have been published ,13,14 ofOther clinical pictures of atypical nystagmus have been described for BPPV, which were explained by the presence of otoconial particles in different parts of the canals. The characteristic they shared was that, in their evolution, after the maneuvers were performed, the typical findings of the affected canal BPPV appeared and were solved with repositioning maneuvers ,10,11,15APV based on epidemiology: pediatric age, except post-HT ,16;APV based on direction of the nystagmus: purely torsional  change in the direction of the nystagmus ;APV based on duration: no latency, excessive duration ,7;APV based on therapeutic test: lack of response to maneuvers ,12,13,14APV based on evolution: persistence of signs throughout time ,6,7,13.Based on the findings of the aforementioned papers, we propose the following elements for a positional vertigo to be considered atypical:Below we present some atypical BPPVs that have been described recently.Calífano et al.  describeIn 2012, Vanucchi et al.  describeWe must differentiate between the more common “canal change” phenomenon, which may be seen when the repositioning maneuvers are not properly performed for the compromised canal, and the more uncommon simultaneous compromise of both canals, which may be observed in cases secondary to head trauma. In these cases, a positional nystagmus with horizontal and torsional components of a magnitude similar to that evoked by the Dix-Hallpike maneuver can be observed; also, a positional upbeat vertical and torsional nystagmus can be obtained with the Dix-Hallpike maneuver to one side, and a positional horizontal direction-changing nystagmus can be obtained with the roll test. The reason for this is that a compromise of the lateral and posterior canal on the same side is the most frequent situation. Compromising of more canals is not frequent .A clinical picture of positional vertigo with upbeat nystagmus has been described, which appears upon the patient sitting up. Scocco et al.  propose Persistent spontaneous nystagmus has been described in lateral and posterior canal BPPV ,20,21,22These alterations, though not totally defined, should be taken into account as theyare the cause of APV, anddo not respond to repositioning maneuvers.A heavy cupula is characterized by the presence of persistent (lasting longer than a minute) apogeotropic positional nystagmus with cephalic changes and of a null point. Some authors have proposed that the density of the cupula would increase as regards the endolymphatic density, thus producing an ampullofugal deflection that would facilitate the persistence of the position-changing apogeotropic nystagmus, depending on the cephalic position. Under normal conditions, the semicircular canals do not depend on gravity, taking into account that the cupula and the endolymph have the same density and therefore the same gravity. However, if the density of the cupula becomes heavier or lighter as compared to that of the endolymph, its deflection due to the presence of otolith remains (debris) alters its gravitational sensitivity. Hiruma et al. hypothesized that a heavy cupula would actually be more of an otoconial phenomenon than a gravitational change and set forth the possibility of a phenomenon in which particles float (“buoyancy”) in the horizontal canal, contrary to what happens in a light cupula, in which there would be an increase of endolymphatic density. In their 2011 paper, they mentioned that patients with a heavy cupula diagnosis responded to repositioning maneuvers, while light cupula patients did not ,24.The light cupula syndrome (LCS) is quite uncommon, but it should be taken into account as it can resemble a horizontal canal BPPV. Patients with LCS usually present with positional vertigo and a constant sensation of imbalance. Nystagmus lasts longer than a minute, is horizontal, geotropic, and direction-changing with head roll. It has no latency or tiredness, showing a constant slow phase velocity that does not fatigue, as is seen in positional alcohol nystagmus phase 1. In the supine position, there is a null point when rotating the head 20 or 30 degrees to the affected side, at which point the nystagmus subsides.The term light cupula was coined by Shigeno in 2002, as it is believed that a direction-changing persistent nystagmus with head rotations is the result of an anti-gravitational deviation of the cupula in the lateral semicircular canal .When the cupula is light, it becomes gravity sensitive, and therefore, when the head is rotated to the affected side, the cupula will be persistently deflected. When the patient is in a sitting position, a spontaneous nystagmus may observed, which will stop when the head is tilted approximately 30 degrees to the front, as this puts the lateral canal in a position parallel to the horizontal plane. In some patients, the light cupula syndrome may be accompanied by unilateral hearing loss, which suggests that there is a concomitant labyrinth alteration. If the nystagmus is apogeotropic, it could be caused by an increase of endolymphatic density. Patients with LCS are refractory to the attempts to reposition the particles. The course of recovery is usually slow, and it takes some days or weeks .Despite the fact that positional vertigo and nystagmus caused by light cupula are similar to BPPV, it has not yet been determined if they are a variant of BPPV. Their pathogenesis is still unknown, and they are generally considered pathologic vestibular phenomena. There are many theories that explain why the cupula becomes lighter than the endolymph but only in the lateral canal, including the following: light debris attached to the cupula; a reduced cupula density as compared to normal endolymphatic density due to an altered homeostasis of sulphated proteoglycans, which are synthesized in the cupula; an increase in endolymphatic density due to chemical changes and a difference between perilymphatic and endolymphatic densities. Light cupula is still a mystery. The nystagmus it presents is similar to that of phase 1 positional alcohol nystagmus, in which the cupula is relatively lighter than the endolymph, as alcohol, which is less dense than water, enters the cupula quicker than the endolymph .The null point is the most important characteristic in the diagnosis of light cupula . The absThe relatively specific change in cupula and endolymph density is dynamic. Light cupula might not be an independent pathology but a pathological condition or stage of an inner ear pathology .To date, there is no effective treatment for light cupula. The existing canalicular repositioning maneuvers do not solve the problem, and more thorough research is needed to find a specific treatment.It is a well-known fact that the cerebellar nodulus/uvula integrates otolith signals for the translational vestibulo-reflex , and thoThere is evidence that shows that the apogeotropic forms are caused by lesions in the nodulus, and that the geotropic ones would be caused by a compromise of the floculus .Its clinical picture is characterized by brief vertigo attacks, which usually last less than a minute and which might occur many times a day. These episodes usually occur as a result of some cephalic movements and are accompanied by hyperacusis and/or tinnitus .We studied 38 patients with a Vestibular Paroxysmia diagnosis (unpublished data) who sought consultation due to spontaneous vertigo. The average age was 59 years.Of the 38 patients, 20 (52.6%) were women and 18 (47.4%) were men.Of the 38 patients, 19 referred positional vertigo, which represents 50%.In addition, 47.3% (18 patients) presented positional nystagmus in the physical examination. In most of these cases, the characteristic was vertical (13 out of 18).Hyperventilation was positive in 28.9% (11 patients).One of the types of vertigo that, according to the Bárány Society, a patient may present during a vestibular migraine attack is positional vertigo .Approximately 65% of patients present positional vertigo  ,36,37,38Central positional nystagmus is present in up to 100% of the attacks, with or without gait ataxia, which is present in 90% of the attacks ,36,37,38Positional nystagmus has a variable pattern: persistent fixed-direction horizontal nystagmus, apogeotropic, downbeating, upbeating, and torsional nystagmus. Interictal may persist after a mild positional nystagmus in the dark ,36,37,38In our database (unpublished data), where a total of 45 patients with a diagnosis of vestibular migraine and with an average age of 56.3 years were analyzed, 13 consulted due to positional vertigo; out of these patients, the following presented positional nystagmus of variable characteristics: eight had down-beat nystagmus, two had horizontal to the left, and two had vertical with a rotational component.Though it is not very common, a clinical picture of vestibular neuritis may produce postural symptoms. We described  a patienIs accompanied by neurological disease signs/symptoms ;Appears during childhood, except post-HT;Presents a purely direction-changing torsional nystagmus ;Has no latency;Is of excessive duration;Does not respond to the maneuvers;Presents signs that persist throughout time.We consider that APV is the positional vertigo clinical picture, whichIn these cases, we must keep in mind that this could possibly be a clinical picture of central origin.We consider that a BPPV is atypical whenThe nystagmus does not fall into the classical description for the affected canal;During its evolution, the typical signs of the suspected canal being affected appear;It responds to repositioning maneuvers;Central causes have been ruled out.We propose the following classification of central positional nystagmus .DEFINITIVE APV means that no maneuver should be applied and that other different diagnoses  should be considered and, if applicable, neuroimages should be indicated.-Purely vertical upbeat positional nystagmus-Purely torsional positional nystagmus-Severe truncal ataxia PROBABLE APV means that maneuvers should be performed and evolution should be followed up.-Though it does not fall into the classical description for the affected canal, the maneuvers result in changes and/or resolution-Geotropic or apogeotropic positional nystagmus that does not respond to the maneuvers, disappears in time, and in which central causes are ruled outIn


Osteochondrolipomas, a very rare combination of chondroid and osseous differentiation within lipomas, are typically found in the neck and head area. We present the case of an osteochondrolipoma in the thigh of a 54-year-old female, with matching histological and cytological correlation. To the best of our knowledge, this atypical location has only been reported once in the literature. Lipomas are the most common benign mesenchymal neoplasm in humans. They usually present as painless, slow-growing masses. History is mostly long but sometimes only recently noticed. Their peak incidence occurs in the 5th to 7th decades of life. Lipomas are found almost anywhere in the body. One can distinguish superficial (subcutaneous) and deep lipomas (muscular tissue) depending on their localization. The latter are further divided into intermuscular, intramuscular, parosteal, or intraosseous. Treatment of choice is a marginal excision, and local recurrence is almost nihil.lipomas are composed of mature adipose tissue, but sometimes they can contain other mesenchymal elements such as fibrous tissue, blood vessels, and, less frequently, cartilage or bone. These variations in composition do not affect treatment nor prognosis [Conventionally rognosis .osteochondrolipoma, which is a mixture of distinct osseous and cartilaginous areas within a bigger adipocytic differentiated mass. Besides this rare combination, its location in the thigh makes it a very uncommon finding. Features and differential diagnosis of osteochondrolipoma are discussed.The present paper reports an unusual example of an A 54-year-old woman was referred to the University Hospitals Leuven with the preliminary diagnosis of a soft tissue tumor in the right upper leg identified as a mass of 43 mm × 62 mm on plain radiograph and echography. She had been complaining of irradiating pain on the lateral aspect of the right leg for 4 months and a one-week history of a palpable mass in the right thigh. There was no remarkable medical history and no recent significant trauma to the affected region.erythema, or other superficial skin lesions were found. The range of motion of the right hip and knee were unaffected and palpation for aberrant inguinal lymph nodes was negative.Clinical evaluation revealed a well-defined nodular mass in the deeper aspect of the right tight, measuring about 5 cm. No concomitant tenderness, swelling, trochanteric region without a sign of bone erosion.At the time of referral, plain radiographs of the hip and pelvis showed crumbly calcification within a soft tissue mass posterior to the myositis (Oedema and adenopathy were absent. Compared to a 2-years old computed tomography (CT) where the mass was overlooked, a minimal volume increase was noted with a current size of 94 mm × 53 mm × 66 mm. The retrospective protocol describes a lesion fully encapsulated by the right musculus quadratus femoris. Biopsy of the lesion demonstrated fibromyxoid to chondroid mesenchymal cells of unknown origin with no evidence of malign morphologic signs.Echography concluded on a non-growing, well-demarcated inhomogeneous muscular tumor without an argument for myositis . Based omyositis . Despitepleomorphic/round cell lipoma was made, and marginal resection was performed. We used the Kocher Langenbeck-type approach with an incision placed slightly more posterior to facilitate exposure and dissection of the sciatic nerve. After muscle splitting of the gluteus maximus fibers with the division of the proximal half of the tendon at 1 cm of its insertions, the sciatic nerve was carefully dissected out and protected. The mass was fully encapsulated by a thin transparent membrane and strongly attached to the underlying femur. The surface of the specimen was stone-hard but smooth, with a yellow shine covering a homogeneous pattern of white and gray dots. The resected tumor was sent for histopathological analysis. Postoperatively, the patient showed no vascular or neurological complications. A mild limp was noted due to dissection of the abductors, which quickly resolved with physiotherapy. At the latest consultation (12 months post-operative), the patient reported manifest improvement of the original discomfort. Clinical examination and ultrasound scans did not show any signs of local recurrence. Plain radiographs did not show any calcified density to suggest local recurrence.Based on this information, a tentative diagnosis of mesenchymal tumor consisting of mature hyaline cartilage islands contiguous with bony trabeculae embedded in mature adipose tissue . This hypothesis has been reinforced over the last years by several studies showing at the first place the presence of different differentiation lines of multipotent stem cells in mature human fatty tissue, and at the second place the in vitro multidirectional differentiation potency of adipose-derived stem cells obtained from liposuction) [lipomas or tumor stroma creating osseous and cartilaginous components).Until now, no clear consensus exists about the pathogenesis of suction) . The secosteochondrolipomas. CT is the procedure of choice to demonstrate the presence of osseous elements and evaluate the relation of the mass to the surrounding bony structures (erosions and/or continuity). 3D reconstruction does not add indispensable information but can help localize the tumor and show the anatomical orientation of the mass, parameters that facilitates excision. In addition, it gives a good overview of the scattered calcifications. On the other hand, MRI has widely considered the best imaging technique for evaluation fatty processes. Lipomas typically appear as a signal identical to subcutaneous fat with a perfect homogenous configuration. The intensity of the signal is stable over all the pulse sequences and will only disappear on the fat suppression sequence. On MRI, calcification and connective tissues will appear as areas with low density.Both CT and MRI are useful in the diagnosis of myositis ossificans, ossifying (fibromyxoid tumor), osteoma, and secondary hyperostosis. Further on, dedifferentiated liposarcoma, osteosarcoma, chondrosarcoma, and teratoma should also be taken into consideration. The final diagnosis will mainly depend on the pleomorphism and atypia of the tumor and the relation of the encountered components to each other . In the same way, needle biopsy only samples an infinite part of the tumor and evaluates tissue architecture or assessment of local invasion impossible. For this reason, incisional biopsy should be favored for comprehensive pathological examination. We also strongly recommend sending the whole mass for further investigation after resection.Although the concomitant presence of osseous, cartilaginous, and adipocytic areas in one specimen is typical for osteochondromalipoma, one should not overlook other pathologies. Cartilage differentiation can be found in soft tissue chondroma , chondroid lipoma, and as part of post-traumatic chondrification. Differential diagnosis of a calcified mass should include osteochondrolipoma located in the thigh of a middle-aged female. Microscopic examination confirmed the presence of cartilage, bone, and fat components within the tumor. After marginal excision, no recurrence occurred at 12 months. Pathogenesis of osteochondrolipoma remains speculative. We reported the case due to its exceptional rarity as being the second described in this region.In conclusion, we exposed the clinical, radiological, and histological findings of an RD, RS, FS, DH, and HW certify that they do not have a financial conflict of interest  in connection with this article.This research did not receive any specific funding.Ethical approval was not required.Written informed consent of the patient was obtained for this publication.RD wrote the manuscript with support from HW and FS. The operation was carried out by HW and RD. The mass was characterized by RS, DH, SF and HW. HW supervised the project.


Monomorphic epitheliotropic intestinal T-cell lymphomas (MEITL) is a rare and aggressive subtype of lymphoma. The most common site of origin is small intestine. Patients are often presented with diagnosis of intestinal perforation with abdominal pain as the main consulting symptoms. Because of the deficiency of specific diagnostic measures and effective management, diagnosis is often confirmed in advanced stage with poor prognosis.Here, we introduce a patient who has suffered from abdominal pain and diarrhea, and eventually been diagnosed as Monomorphic epitheliotropic intestinal T-cell lymphomas.MEITL is rare in clinical practice with deficiency of early diagnostic measures and poor prognosis. Therefore, any patient with ambiguous gastrointestinal symptoms or perforation of the digestive tract where the primary lesion is difficult to identify should be alert to the possibility of this disease. The gastrointestinal tract is the most common origin of extranodal lymphoma and accounts for 30–40% of all cases. Enteropathy-associated T-cell lymphoma (EATL) is divided into two types: type I EATL and type II EATL. Type I EATL, typically characterized by celiac disease, is common in Western countries. Type II EATL, characterized by abdominal pain and intestinal perforation, is more common in Asia . AccordiMEITL, a very rare subtype of lymphoma, is involved in less than 5% of lymphoma of the digestive tract. It occurs worldwide with predominance in Asia, and is less common in Western populations. According to the report, the incidence of the elderly is higher with a median age of 58 years in the largest series reported to date . It usuaAmong all pathological subtypes of lymphoma, B-cell lymphoma is most common which accounts for 90%. In contrast, T-cell lymphoma is rare . MeanwhiAs for the clinical presentation, the common symptoms of MEITL include abdominal pain, malnutrition, intestinal obstruction, acute abdominal pain, and even intestinal perforation . HoweverA 49-year-old man was admitted to LongHu Hospital, the First Affiliated Hospital of Shantou University Medical College with a history of abdominal pain and diarrhea for 7 days. The pain mainly occurred around the umbilicus, presenting in the manner of colic pain. The patient also complained about generalized fatigue and low-grade fever.At admission, the body temperature was 38.2 °C, with the pulse 116 beats/min and blood pressure 105/64 mmHg. Physical examination revealed that breath sounds of both lungs were thick and the abdomen was flat with tenderness around the umbilicus, without rebound tenderness or other positive sign.In history taking, a colonoscopy which revealed ulcer in ascending colon and rectum polyp was performed in the Shantou Central Hospital before admission. Pathological examination revealed chronic inflammation of ascending colonic mucosa accompanied by erosion, and rectal tubular adenoma.After admission, the patient experienced the pain worsening than before. The pain gradually disseminated to the whole abdomen, presenting persistent colic. Physical examination found tabular abdomen, obvious tenderness and rebound tenderness in the whole abdomen, with reduced bowel sounds. The computed tomography (CT) indicated perforation of digestive tract (most likely in the splenic curvature of the colon) and massive effusion in the abdominal cavity. Therefore, urgent laparoscopy surgery was performed. Intraoperative exploration found no obvious perforation, which could not explain the patient’s peritonitis. After cautious consideration, terminal loop ileostomy was performed in view of potential obscure colonic perforation. With thorough intraoperative exploration, it was found that the serosa of the appendix was crackable and the canal wall was stiff. An appendectomy was performed accordingly and four drainage tubes were placed after thorough abdominal irrigation. The stable postoperative vital signs and gradually reduced drainage fluid indicated smooth recovery.At histological examination, the diffusely infiltrating lymphocytes at the appendix wall was uniform, with various sizes, coarse-grained chromatin and unclear nucleoli. Epidermophilic phenomena could be seen and lymphoma could not be ruled out Fig. . The micImmunohistochemistry reveals cell positivity for the following markers: CD3, CD7, CD8, CD56, TIA-1, Ki-67, CD4, while CD5, CD20, CD30, TDT, ALK and EBER are negative  showed intestinal obstruction with multiple free gases in the abdominal cavity. Abdominal paracentesis and catheterization were performed for drainage and irrigation. The drainage fluid was cloudy dark green with more gas visible in the drainage bags. After treatment with broad-spectrum antibiotics, intravenous fluid resuscitation, analgesics, antiemetics, and antithrombotic drugs, the patient showed no sign of recovery. Subsequently, due to worsening infection, the patient gradually developed thrombocytopenia, coagulation dysfunction, electrolytes imbalance and finally died from multiple organic dysfunction half a month later.According to published reports, Lymphoma accounts for only a small fraction of all gastrointestinal malignancies with a proportion of about 1–4%. Primary lymphoma of the appendix is rare of all tumors origining from the digestive tract. It presents symptoms of acute appendicitis or acute abdomen.In this case, the initial symptoms presented were abdominal pain and diarrhea on admission. The patient bore a primary T-cell lymphoma with immunophenotype features consistent with monomorphic epitheliotropic T-cell lymphoma. MEITL, formerly known as type II EATL, is a primary intestinal T-cell lymphoma caused by malignant proliferation of intraepithelial lymphocytes . It is cIt is reported that small intestine is the most commonly affected part of MEITL, especially jejunum and ileum, and then in order duodenum, stomach, and colon. In our case, the source of the lesion is appendix.Due to the unspecific clinical manifestations, most cases are not diagnosed until the neoplasm reaches advanced stage. Common laboratory tests and imaging scans were inconclusive. The best approaches to diagnosis are endoscopic biopsy and surgical pathology. Pathological and immunohistochemical results are essential for diagnosis. MEITL is usually positive for CD3, CD8 and CD56, but negative for CD5, CD30 and CD103, with small to medium-sized cells [Because of the aggressive progression of the disease, the prognosis of MEITL is generally poor . Therefo


In this study, we present an eight-year-old patient with intraosseous hemangioma in the right humerus diaphysis. The humerus diaphysis is an unusual localization for hemangioma. To our knowledge, this is the first case of intraosseous hemangioma in the humerus diaphysis in a pediatric patient.Treatment of intraosseous hemangiomas is controversial; options range from untreated follow-up to en-bloc resection. Intralesional curettage and grafting with cortico-cancellous allograft were performed in this case. Around 22 months postoperatively, she showed full shoulder and elbow function and there was no evidence of local recurrence or metastasis. Intraosseous hemangiomas (IHs) are rare benign vascular tumors and account for less than 1% of all bone tumors. In Turkey, there are publications reporting that it is seen less than one percent . LesionsMost bone hemangiomata are seen on radiographic findings with the formation of reactive spicules produced by the lesion . ClinicaBecause of the variable appearance of the lesion, histological examination is vital to obtain a definitive diagnosis. Other benign and malignant tumors  should be considered in the differential diagnosis .There are only a few case presentations reporting hemangiomas localized in the humerus. Moreover, humeral diaphysis is a very rare localization ,6,8,9. WAn eight-year-old girl was admitted to our orthopedics department with a complaint of pain in her right arm midline. She said her complaints had been going on for six months and getting worse. In addition, the patient could not recall any trauma and did not have any systemic or metabolic disease. The study was conducted in accordance with the principles of the Declaration of Helsinki.On the clinical examination, pain was detected in the midline of the right arm, but shoulder and elbow joint movements were regular and painless. On the plain radiograph, there was a smooth lesion with a narrow transitional zone and foamy patchy lytic areas in the humeral diaphysis leading to expansion of the central bone. Within the lesion, a linear lucent line was observed, which was consistent with the fracture leading to a smooth periosteal reaction, extending obliquely to the medial and lateral cortex Figures -1B.The MRI revealed a 72 × 21x21 mm mass in the right humeral diaphysis with an intramedullary expansile nature. In T1 and T2 MRI-weighted series, the heterogeneous density of the mass and contrast enhancement after intravenous gadolinium injection are noteworthy. In addition, thinning of the cortex, patchy irregularities, and defective view suggested pathologic fractures. In addition to the described lesion, contrast enhancement was observed in the soft tissues after intravenous injection of contrast material in the posterolateral sections Figures -1C.Incisional biopsy was performed and intraosseous hemangioma was shown. Histological examination of the excised tissue confirmed the typical features of the hemangioma Figures -2C. The No intraoperative or postoperative complications were observed. At 24 months of follow-up, the patient did not feel any pain and there was a full range of motion in the shoulder and elbow.IHs constitute <1.0% of all bone tumors; moreover, 1. 75% of tumors are localized in the vertebral body and skull. In 15-20% of all cases, tumors are found in the scapula, ribs, clavicle, and pelvic bones ,10. The IHs are typically seen in adult patients in the third and fourth decades of life, thus very rarely seen in skeletally immature patients younger than 10 years. In their literature review, Andreciccio et al. found that there were 14 cases, including his own, in 2007 [Although IH is the most common solitary lesion, approximately 18-25% of IH cases are multifocal . IH withPlain radiographs show a classical coarse trabecular bone pattern or soap bubble appearance. Most lesions show cortical destruction . On plaiRadiographic diagnosis of an atypical intraosseous hemangioma is more difficult due to various possible diagnoses such as fibrous dysplasia, solitary or aneurysmal bone cysts, eosinophilic granuloma, multiple myeloma, chondrosarcoma, Ewing’s sarcoma, metastasis, other benign and malignant lesions .The biopsy will be the gold standard to obtain the correct diagnosis . Needle Histopathological examination of intraosseous hemangioma reveals thin-walled blood vessels. If these structures are damaged during biopsy or curettage, non-diagnostic empty spaces may be encountered and only bone trabeculae are detected. Therefore, an intraosseous hemangioma may pose a diagnostic challenge for the pathologist 3. In our study, there were sections containing bone trabeculae, and the diagnosis was made by the presence of dilated thin-walled vascular structures containing erythrocytes. The differential diagnosis of an aneurysmal bone cyst was made with the absence of cellular blood-filled spaces and fibroblastic cells.The treatment of intraosseous hemangioma is a controversial subject ranging from biopsy to segmental resection ,11. SmalThis is the first report of an intraosseous hemangioma of the humeral diaphysis in a pediatric patient. After obtaining the histopathological diagnosis by biopsy, it was successfully treated with curettage and bone grafting.


Rapidly destructive coxarthrosis is a rare entity of unknown etiology that is characterized by rapid hip joint destruction. Blood tests are thought to be non‐specific. However, we herein show a patient with rapidly destructive coxarthrosis, which was accompanied by fluctuating C‐reactive protein level. Rapidly destructive coxarthrosis should be in the differential diagnosis for acute hip pain with an elevated CRP level, even if the joint deformity is slight on radiography, and early surgery should be considered. A 74‐year‐old woman presented with a 3‐day history of left hip pain. Her left hip joint exhibited tenderness, and pain was induced by rotation. A CRP level was elevated (11.83 mg/dl). Radiography of the left hip slightly demonstrated joint space narrowing Figure . SynoviaRapidly destructive coxarthrosis is a rare entity of unknown etiology. Initial presentation is acute hip pain without radiographic evidence of joint destruction. Complete vanishing of the proximal femur abruptly occurs within a few months.The authors declare that they have no competing interests.HS collected the data and wrote the first draft of the manuscript. HN coordinated the project and edited the manuscript. Both authors have read and approved the final manuscript.Written informed consent was obtained from the patient. This case is anonymous.


Angioleiomyoma is a benign neoplasia originating from vascular smooth muscle and very uncommon in the oral cavity. In this report, we present a rare case of angioleiomyoma in oral cavity in a 46-year-old female buccal cheek and discuss the clinical, histological, and immunohistochemical characteristics. As the treatment of choice is the complete excision, the lesion was excised under local anesthesia with no further complications. In addition, a brief update on other reported cases of angiomyoma in the oral cavity is further discussed. Angiomyoma or angioleiomyoma  are benign neoplasms originating from vascular smooth muscle , 2. HistA 46-year-old female was referred to a private clinic of an Oral and Maxillofacial Surgeon with complaint of a painless swelling in the right side of her cheek since 6 years ago. The swelling was initially small in size and slowly enlarged, although the patients faced no tenderness in the area. The patient had diabetes mellitus and anemia. In addition, no history of previous trauma to the area was present. Extraoral examination showed no asymmetry or marked expansion on the right cheek. On intraoral examination, no clinically visible lesion was detected. The mucosa was intact with no color change. When the area was palpated, a firm, compressible solid mass was detected with moderate attachment to the adjacent tissues. The estimated size of the lesion was 1.5∗1 cm, and the approximate diameter was 1.5 cm. No pulsation or bruit was present. The patient had previously visited and had prior imaging including Doppler ultrasonography, multidetector computed tomography (MDCT), and MRI. The results of ultrasonography indicated a well-defined hypoechoic heterogeneous mass lying beneath the skin measuring 13∗18 mm . The lesAngioleiomyoma is a rare benign soft tissue neoplasm of oral cavity . AlthougPreoperative radiologic assessments varied among the studies, from no radiographic assessments and plain radiography to ultrasound, MRI, and CT evaluations. To our knowledge, this is the first case report of angioleiomyoma with a thorough imaging assessment including color Doppler sonography, MDCT, and MRI. The lesion was slightly heterogeneous hypoechoic mass with slight vascular flow in the color Doppler sonography; in addition, the T1-weighted and T2-weighted sequences were low and high, respectively. The high T2-weighted sequence may be seen in cysts, benign or low-grade minor salivary gland tumors, and rare hemangiomatous lesions . In thisIn this case, H&E histologic examination showed a tumor consisting of thin- and thick-walled blood vessels in a background of smooth muscle proliferation. Having numerous blood vessels may pretend other benign vascular tumors such as hemangioma, hemangiopericytoma, hemangioendothelioma, vascular malformation, and other neurovascular hamartomas , but a dIn all evaluated cases, the lesion was resected with an excisional biopsy under local or general anesthesia. There are no recurrences after resection.In conclusion, we have reported a case of angioleiomyoma of the buccal cheek that resulted in a good outcome. Among various studies, the most common sites of oral angiomyomas were the lip –15, 32,


Case Presentation. We report the case of a 70-year-old woman presenting an epigastric swelling. Radiological findings showed a liver herniation in a primary ventral hernia. This case is the first to have been described requiring semiurgent hernia repair associated with partial liver resection.  Herniation of the liver through the anterior abdominal wall is an extremely rare phenomenon. Most cases occur within an incisional hernia (mostly upper abdomen surgery or cardiac surgery). Only two reports mentioned liver herniation without previous abdominal incision.  This case is, to the best of our knowledge, the first case of primary ventral hernia with liver content necessitating wedge resection of the left liver lobe. Herniation of the liver through the anterior abdominal wall is an extremely rare phenomenon. Most cases of liver herniation are due to diaphragmatic hernias . In a minority of cases of the childhood, the congenital anomaly is an abdominal wall defect with omphalocele .In adults, hepatic herniation through the anterior abdominal wall is a really rare entity. Most occurred within an incisional hernia secondary to supraumbilical laparotomy for supramesocolic surgery  or abdominal extension of sternal incision  , 3. OnlyThis case is the first to describe semi-urgent hernia repair associated with partial liver resection. Herein, we discuss diagnosis and surgical management of this rare case.We report the case of a 70-year-old woman who was brought at the emergency service for evaluation of a sudden epigastric pain and nausea. She denied any history of trauma or heavy lifting. Her past medical history included total hip replacement, caesarean section, and a femoropopliteal artery bypass. She had no previous abdominal surgery history. She smoked for over 55 pack years.3 with presence of a discrete inflammatory syndrome (CRP 47 mg/L), and hepatic cytolysis ranged twenty times the normal values .Clinical examination revealed an important unreductible epigastric swelling. Biologically, haemoglobin was normal (Hb 13.3 g/dL), WBC count reached 20800/mmAn abdominal CT scanner showed huge epigastric hernia containing the left liver lobe associated with small amount of ascites within the hernia sac Figures  and 2. APostoperative course was marked by an important ileus requiring a nasogastric tube for five days.In adults, liver herniation through the abdominal wall is a very scarce phenomenon. Most cases occurred within an incisional hernia (mostly upper abdomen surgery or cardiac surgery) , 3.Two major questions arouse while performing the surgery: whether to resect the left liver lobe and whether to place or not a mesh.Liver parenchyma is well vascularized by the hepatic artery and portal vein. Three elements in the case presentation were in favor of acute liver low perfusion of the incarcerated part: segment III hypodensity on injected CT scan, important inflammatory response with high WBC, and cytolysis edging twenty times the normal range.Moreover, transient arterial ischemia can result in ischemic cholangiopathy . FurtherThe rationale for performing a liver resection and for mesh placement in this case was put into balance with the contamination risk due to ischemic cholangiopathy or a liver abscess and with the recurrence risk with no mesh use.It is now widely accepted that mesh repair for incarcerated or strangulated hernias is feasible with a great benefit of lower recurrence rates . The useFor all these reasons, the ventral hernia cure using a mesh and partial left liver lobe resection were performed.This case is, to the best of our knowledge, the first case of primary ventral hernia with liver content associated with wedge resection of the left liver lobe.


Intussusception in healthy adults is rare and often associated with oncologic diseases. This case report presents a case of an ileo-colic intussusception reaching down to the descending colon in a healthy adult that required ileo-colic resection.We present a case of a 78-year-old male patient with acute onset unspecific abdominal pain. The medical history was unremarkable. Preoperative radiologic assessments showed an invagination of the small intestine into the colon without any signs of polyps or tumours. An emergency laparotomy with resection of the affected intestine was performed. The pathologist described a 49 cm length of intussuscepted colon and an additional 7 cm intussusception of the terminal ileum. A circular area with multiple polyps extending over 8 cm in the colon could be identified. The microscopic findings showed a low-grade dysplasia within this area. Following surgery, the patient was discharged to rehabilitation after a ten-day hospitalization.Intussusception in adults is rare and the clinical presentation includes unspecific symptoms making the diagnosis challenging. In 90% of the cases, a pathologic lesion is found (two-thirds are neoplasms). An intussusception involving the colon should be treated surgically without prior reduction due to the high incidence of a neoplasm and the risk for perforation and tumour dissemination.In the literature, neoplastic disease represents the major cause for intussusception in adults. This report presents a rare case of an ileo-colic intussusception reaching down to the descending colon treated successfully with a subtotal colectomy. •Intussusception is a differential diagnosis in adults with abdominal pain.•In up to 90% of the cases, an underlying disease or condition can be identified.•Increasing incidental diagnosis due to better quality and availability of CT scan.•If the colon is involved, the best treatment is surgical without prior reduction. He denied having had fever or any history of gastrointestinal bleeding. Since the onset of his complaints, the patient had loss of appetite. His weight was stable in the last year. Otherwise, the medical history, including genetic information, was unremarkable and the patient was under no regular medication. The family history, as well as the psycho-social history, were unremarkable. The patient never had a colonoscopy nor had he ever undergone abdominal surgery before. No further relevant interventions were reported.Examination of the abdomen showed a periumbilical palpable mass extending towards the right lower quadrant. The mass was not tender, and there were no sign of peritonitis. Laboratory testing provided slightly elevated inflammatory markers and negative tumour markers. The following computed tomography (CT) showed a pronounced invagination of the small intestine and the colon , Fig. 2.The patient was prepared for emergency explorative laparotomy, which was performed by a senior consultant. Intraoperatively, we found an ileo-colic intussusception that could be palpated down to the descending colon. There were no signs of intestinal ischemia, peritoneal carcinosis, or suspicious mesenterial lymph nodes. The colon wall was fragile and at risk of tearing during the manipulation. We decided to perform a colectomy of the affected colon and the terminal ileum. This resulted in a subtotal colectomy with en-bloc resection according to oncological principles. The bowel continuity was restored with a side-to-side anastomosis of the terminal Ileum and the descending colon, and the abdominal wall was closed in the usual manner. Postoperatively, the patient underwent gradual progression from liquids to solids and was discharged after ten days of hospitalization.In the histopathological examination, the pathologist macroscopically described 49 cm of intussuscepted colon with a diameter of 4 cm . The eme3This case presents a rare example of an ileo-colic intussusception reaching down to the descending colon in an otherwise healthy patient who presented with unspecific abdominal pain. Based on the palpable intraabdominal mass and the patient's age, we suspected a growing tumour. Some differential diagnoses were also taken into consideration. An acute appendicitis, especially in elder patients, might present with similar symptoms, and the absence of fever does not rule out an appendicitis The anatomical presentation of an intussusception varies widely: gastro-duodenal, jejuno-jejunal, ileo-ileal, ileo-colic, and colo-colic. In 90% of cases, a pathologic lesion is found—most of them intraluminal The paediatric patients with intussusception can present with the triad of acute onset colicky pain, palpable abdominal mass, and—with disease progression—bloody stool. This triad, however, is described in less than 15% of cases Plain radiograph may reveal signs of obstruction and its location, but they lack sensitivity and specificity for the diagnosis of intussusception Regarding the best treatment, the literature remains controversial due to the different aetiologies in children and adults. In the literature, adult intussusception has been primarily treated surgically with resection of the affected bowel segment due to the prevalence of a malignancy as a leading point If a benign lesion has been diagnosed and the bowel is viable or the resection will lead to short gut syndrome, one should consider a preoperative reduction. A colonoscopy can also be pursued to confirm the pathology or malignancy In our case, the patient was diagnosed with an ileo-colic intussusception, and we performed an emergency laparotomy and resected the affected bowel segment, following the oncological principles. In the histopathologic findings, there was evidence of a circular area extending over 8 cm with multiple polyps as a leading point for the invagination with microscopical evidence of a small area with low-grade dysplasia without lymphatic metastasis and negative resection margins. There were no postoperative complications during the stay at the hospital or after discharge.As a learning point, we believe that the description of this case, alongside review of the literature, may not only raise awareness to intussusception as a differential diagnosis on a setting of abdominal pain but also help keep in mind that even in such a case the basic principles of surgery are applied.4Elderly patients presenting with abdominal mass, sub-ileus symptoms, and loss of appetite would raise the suspicion of a malignant disease, especially in patients who did not undergo prior routine screening for colon cancer. However, the rare diagnosis of intussusception represents a valid differential and should be considered in patients with these symptoms.Not commissioned, externally peer-reviewed.No external funding was received for this study.This case report is exempt from ethical approval in our institution.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal upon request.Hugo Teixeira treated the patient and wrote the original article. Fabian Hauswirth and Nina Römer treated the patient and critically reviewed the article; Markus K. Muller critically reviewed the article; Matthias Baechtold treated the patient, supervised and critically reviewed the article.Not applicable.Hugo Teixeira.None of the authors declared any conflicts of interest.


Gallbladder carcinoma (GBC) with proficient mismatch repair (pMMR)/microsatellite stable (MSS) is associated with limited response to programmed death-1 (PD-1) inhibitor monotherapy. Limited data of PD-1 blockade combined with anti-angiogenic therapy in GBC are reported. One recurrent GBC patient with pMMR/MSS was treated with camrelizumab plus apatinib. After 4 cycles of combination therapy, the patient achieved a durable complete response with manageable toxicity. The next-generation sequencing and immunohistochemistry analysis showed that tumor mutation burden (TMB) was 7.26 mutants/Mb and PD-L1 expression was 10% (tumor proportion score) and 20% (immune proportion score). This case suggests that camrelizumab in combination with apatinib may be an effective treatment option for GBC patients with pMMR/MSS status, who have moderate expression of TMB and PD-L1. Additionally, TMB and PD-L1 expression may serve as potential biomarkers for predicting PD-1 inhibitor response of GBC. Furthermore, this needs to be verified in future studies. Gallbladder carcinoma (GBC) is one of the most common primary malignancies of the biliary tract. Treatment protocols for advanced GBC are limited, and the prognosis is extremely poor, with a median overall survival of around 4–7 months . At presvia relieved hypoxia, accelerated tumoral infiltration of CD8+ T cells, and decreased recruitment of tumor-associated macrophages (Immunotherapy such as immune checkpoint inhibitors (ICIs) had become prospective therapeutic schemes in a variety of cancers. Several clinical trials were also conveyed in biliary tract cancers (BTC), including the KEYNOTE-028 trial and KEYNOTE-158 trial. However, the objective response rate (ORR) of ICI monotherapy are only about 6%–13% in BTC , albumin-bound paclitaxel , and trastuzumab  were administered every 3 weeks.The patient was a 60-year-old male with a medical history of hypertension and hepatitis B cirrhosis. He received regular anti-hypertension and antiviral treatment. In May 17, 2020, abdominal computed tomography (CT) revealed a gallbladder mass at the fundus (2.7 × 2.2 cm in size) at an annual medical examination. Enhanced scan showed that the mass was markedly enhanced. Enlarged lymph nodes were not found in the abdomen. He showed no obvious symptoms of discomfort, and physical examination was normal. Then, he received open cholecystectomy and regional lymphadenectomy in May 27, 2020. Surgeons found that the tumor directly invaded the liver. Therefore, hepatectomy with partial resection of segments 4 and 8 was performed. Postoperative pathology indicated a pT3N0M0 adenocarcinoma with poor differentiation, without lymph node metastasis and with a negative surgical margin. The abundance of ERBB2 mutation was 26.94%, which suggested the possible therapeutic potential of trastuzumab. Then, he was treated with 6 adjuvant treatments from July 18, 2020, to October 18, 2020. Gemcitabine  indicated multiple lesions in the right lobe of the liver, the largest measuring 1.1 cm × 1.1 cm adjacent to the surgical zone Figure 2On December 22, 2020, MRI showed exceptional shrinkage of the tumor and the lesion under the liver capsule of segment 5 (S5) disappeared, with a partial response after two cycles of treatment  can be found in the following: NCBI SRA BioProject, accession no: PRJNA786280.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.LZ: manuscript writing. XL and ZL: imaging analysis. XZ and YW: data collection. WP: manuscript revision. All authors contributed to the article and approved the submitted version.This work was supported by the Health Science and Technology Project of Jiangxi Province (202140765).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Traditionally, caecal volvulus (CV) and sigmoid volvulus (SV) have been thought of as largely separate clinical entities with distinct clinical features, radiological findings and treatment strategies. We present a rare case of synchronous CV and SV. To our knowledge, this represents only the ninth such case in the literature. This posed a diagnostic challenge as the seemingly textbook features of SV, such as the classical ‘coffee-bean’ sign on plain abdominal X-ray, masked the simultaneous occurrence of CV which only became apparent after the patient continued to deteriorate despite the successful endoscopic decompression of the SV. The diagnosis of SV should be made cautiously, with a period of close clinical observation post-intervention and a low threshold for re-evaluation should symptoms persist or recur to ensure accurate diagnosis. Volvulus is the axial rotation of a portion of the gastrointestinal tract along its supporting mesentery . This moThe simultaneous occurrence of CV and SV is an exceedingly rare clinical entity, with less than 10 cases reported in the literature to date . For thiA 54-year-old female presented to the emergency department with a 2-day history of crampy abdominal pain, distension and absolute constipation. She had a history of chronic constipation but no other significant medical history. Her abdomen was diffusely tender and grossly distended on examination, with abdominal X-ray showing the ‘coffee bean’ appearance of SV. Computed tomography (CT) of the abdomen was performed which confirmed the presence of SV without evidence of ischaemia or perforation . CV was The patient continued to experience abdominal pain with multiple episodes of vomiting post-procedure. A wide-bore nasogastric tube was placed which drained 850 ml of bilious fluid. New onset tachycardia was noted and physical examination raised concerns for peritonitis. A further abdominal CT was performed which showed that the SV had been successfully decompressed but also revealed the presence of CV with proximal small bowel dilatation . There wA midline laparotomy was performed. A CV was found with necrosis of the caecum, which was highly mobile . The SV et al. [A synchronous double volvulus of the colon affecting the caecum and sigmoid colon concurrently is an exceedingly rare condition. A detailed literature search revealed less than 10 previously reported cases  Table 1Table 1. et al. , CV was The underlying aetiology of colonic volvulus has long been debated, with a frequently proposed theory being that the stretching and lengthening of the mesentery occurs as a result of long-standing constipation . It is aOur case highlights a number of important learning points. The first is that the diagnosis of SV should be made cautiously, with a period of close clinical monitoring post-intervention being critical. Any subsequent deterioration or recurrence of symptoms should prompt thorough re-evaluation to ensure accurate diagnosis. The second is that CV and SV may not be truly distinct clinical entities as they have previously been described, potentially arising from the same underlying aetiology, and thus, patients that develop one may be at risk of the other. For this reason, it is worthwhile to outrule the presence of synchronous volvulus during surgery for what is initially suspected to be an isolated SV or CV. Finally, in the unusual case of concurrent SV and CV, the definitive surgical treatment is a total colectomy, with or without anastomosis depending on patient status, allowing for both pathologies to be treated. While there may be an argument to be made for organ-preserving techniques, such as sigmoidopexy or repeated endoscopic decompression, it would appear that the consensus from previously reported cases is that total or subtotal colectomy is the favoured surgical approach. This offers the advantage of eliminating the risk of recurrence while also allowing for histopathological analysis to outrule underlying malignancy. In many of these previously reported cases, patients had presented with symptoms on multiple occasions despite previous endoscopic management, highlighting the need for a definitive surgical solution.None to declare.No funding received.Obtained from the patient and documented in the medical notes.


Bezold’s abscess (BA) is a severe and rare extracranial complication of suppurative acute mastoiditis. The diagnosis of BA requires a high index of suspicion due to its rarity. In this study, we present a rare case of BA, in addition to a review of literature over 20 years. We searched for all cases in English literature from 2000 to 2020 in PubMed and found 27 cases (28 cases including the current case). BA was more prevalent in males  and adults . Of the 28 cases, six were associated with cholesteatoma and another six cases occurred with concomitant sinus thrombosis. Bezold's abscess (BA) is caused by pus draining through the medial wall of the mastoid process and producing a suppurative collection in the digastric sulcus . This abA 46-year-old male presented to our emergency department complaining of left otorrhea, high fever, left post-auricular pain, and a painful left neck swelling, which increased in size over the past four days with decreased hearing. The patient had a history of right otorrhea periodically for three years that was treated multiple times with oral antibiotics and local antibiotic ear drops. In addition, the patient had a history of Bell’s palsy three years previously, which resolved with medical treatment and facial exercise. The patient denied a history of vertigo, tinnitus, airway symptoms, dysphagia, odynophagia, or trismus. There was no history of previous ear surgery.On examination, the patient was febrile; he experienced pain, but his vital signs were stable. There was post-auricular erythema and a left lateral diffuse swelling over the mastoid bone extending down to the upper sternocleidomastoid muscle with tenderness and fluctuation Figure . On otosComputed tomography of the head and neck with contrastThere was evidence of acute mastoiditis with bony erosive changes in the left mastoid bone and a thick walled peripherally enhancing radiolucency measuring 3.5 x 2.6 x 3.5 cm in the left retrosternal region abutting the sternocleidomastoid muscle posteriorly  ceftriaxone 2 g and IV paracetamol 1 g were started immediately. On admission, ceftazidime 2 g and metronidazole 500 mg were prescribed. The next day, the patient underwent a cortical mastoidectomy, and an incision and drainage of the abscess under general anesthesia was performed. After the incision, copious pus came out, which was sent for culture Figure . The resBA is a severe and rare extracranial complication of suppurative acute mastoiditis. We searched English literature for all the cases reported from 2000 to 2020 in PubMed. We found 28 cases (including ours), as presented in Table The diagnosis of BA requires a high index of suspicion due to its rarity. Patients may not show any signs of sepsis, and clinicians should be aware of this complication in patients with otitis media or acute mastoiditis. The most prevalent reported clinical signs and symptoms were fever, otalgia, neck edema, neck pain, otorrhea, torticollis, facial paralysis, and hearing loss . Our patThe mastoid tip, which is pneumatized in adults, is composed of thin-walled air cells. The lateral wall of the mastoid is composed of thicker bone than that of the medial wall. The lateral wall serves as an insertion site for the sternocleidomastoid, digastric, splenius capitis, and longissimus capitis muscles. Pus in the mastoid erodes through the mastoid tip, the area of least resistance, which is inferior and medial. The abscesses are formed deep in the neck muscles, which delay its detection. Another factor that could contribute to the delayed diagnosis is the unfamiliarity of the disease to the clinician.BA has been linked to lateral sinus thrombosis , which iThe diagnosis can be challenging because of rarity of this abscess and the variable signs and symptoms. Computed tomography (CT) and magnetic resonance imaging (MRI) images can locate the abscess, as described in Table Streptococci species the major pathogens. In our review, Streptococci were the most frequent causative organisms. However, multiple organisms, both gram positive and negative, as well as anaerobes, were cultured. Antibiotics effective against gram-positive organisms should be initiated since they are the most frequent causative pathogens. Subsequently, culture-based antibiotics can be described. In addition to the IV antibiotics, a surgical intervention (mastoidectomy and abscess drainage) is required for the effective management and prevention of further complications. Of the 28 patients with data regarding treatment, 21 (75%) underwent a mastoidectomy . In almost all the cases, the gold standard management was IV antibiotics, drainage of the abscess, and mastoidectomy.


Trichotillomania and trichophagia cause trichobezoars, which are masses made of hair. The main presentation of this condition is abdominal pain. However, other complications include gastric outlet obstruction, nausea, vomiting, weight loss, malnutrition, hematemesis, diarrhea, and constipation.A 57-year-old woman with trichotillomania was admitted to the Emergency Department with the chief complaints of dyspnea on exertion, shortness of breath, dysphagia, generalized weakness, and hoarseness. Spiral chest computed tomography (CT) scan did not reveal any parenchymal lesions Pulmonary CT angiography did not reveal pulmonary embolism. The patient was admitted to the Surgery Department for hand fasciotomy due to contrast leakage, and during laryngoscopy, a trichobezoar was detected that was removed with Magill forceps.Rare cases of trichobezoars can be observed in humans with gastrointestinal and respiratory symptoms. Precise and timely diagnosis are key for the prevention of more invasive diagnostic procedures. Trichobezoars are defined as the accumulation of hair in the stomach secondary to impulsive hair-pulling and consumption. Trichobezoars may present in both children and adolescents with a history of trichotillomania and trichophagia . In the The presentation of bezoars in humans was first reported in 1779. The patient had expired due to gastric perforation and peritonitis, and the patient’s autopsy revealed a bezoar. Bezoars may be made of vegetable or fruit fiber (phytobezoars), milk curd (lactobezoars), or any other indigestible material; however, the most common type of bezoar in humans is trichobezoar made of hair. In a rare case named Rapunzel syndrome, the trichobezoar may include a tail of hair stretched from the stomach into the duodenum and jejunum , 3.The most common clinical complications of patients with trichobezoars include chronic abdominal pain, palpable abdominal mass, gastric outlet obstruction, nausea, vomiting, weight loss, malnutrition, food intolerance, hematemesis, diarrhea, and constipation .Due to the high prevalence of long hair in women, trichobezoar is more common in the female population. The mean age for trichobezoars in women is reported to span from 13 to 20 years old. So far, some cases of cotton bezoar with Rapunzel syndrome, and in some rare cases, bezoars in the stomach and intestines have been reported. In this report, we aimed to report a rare case of trichobezoar in the subglottic area detected during laryngoscopy.A 57-year-old woman with a history of hypertension, diabetes, major depression, and trichotillomania was admitted to the Emergency Department with the main complaints of dyspnea of exertion, shortness of breath, and generalized weakness that lasted for 3 weeks Fig. a. She al2sat = 75% with FiO2 = 21% and O2sat = 89% with FiO2 = 50%. The differential diagnosis consisted of obesity hypoventilation syndrome, obstructive airway disease, and pulmonary thromboembolism. Spiral chest computed tomography (CT) scan did not reveal any parenchymal lesions. Venous blood gases  and d-dimer > 500 were found on clinical evaluation.The vital signs were as follows: HR = 112, RR = 19, BP = 135.87, OAfter evaluation, we suspected pulmonary embolism, and we performed pulmonary CT angiography. After the injection of a contrast agent, the patient suffered a leakage of the contrast agent from the vein under the skin, causing swelling of the hand Fig. b.2 = 53, HCO3 = 28.7). In follow-up, the patient underwent endoscopy for the evaluation of esophageal abnormalities, which was normal, only a few hair strands were seen in the esophagus.With the diagnosis of hand compartment syndrome, the patient was taken to the operating room for a hand fasciotomy. She was not well ventilated using a laryngeal mask airway (LMA). Due to the lack of ventilation, the decision was made to intubate. During laryngoscopy, a foreign body in the subglottic area was detected Fig. c. We remTrichobezoars are attributed to the accumulation of foreign masses in some parts of the gastrointestinal system. Epidemiological findings indicate the higher incidence of trichobezoars in older girls and adult women with psychiatric illnesses and trichophagia. In a study conducted in trichobezoar patients between 18 and 26 years old, female to male ratio was achieved 4:1 and 15:1 in younger adults and older patients, respectively , and (c)We report a 57-year-old woman who presented with shortness of breath, and during laryngoscopy was fund to have a trichobezoar in the subglottic area. In reviewing related case reports regarding trichobezoars, cases similar to the one described in this study are less common. Therefore, this case is reported to highlight that in patients with a history of trichotillomania and trichophagia, some rare and unwanted problems may occur. Therefore, in patients with trichotillomania with unexplained hypoxemia, bezoars in the unusual site such as subglottic area should be considered as a probable diagnosis.


The Fontan procedure (FP) is a palliative surgery for functional single ventricle. The Fontan circulation maintains pulmonary circulation by a high central venous pressure, leading to chronic congestive liver. The number of patients diagnosed with hepatocellular carcinoma (HCC) arising from liver fibrosis and cirrhosis after FP is increasing. Several reports have described surgical treatment for HCC after FP, but few have described laparoscopic surgery.The patient was a 31-year-old man who had undergone the FP for single right ventricle at 3 years. Several liver masses were detected at 30 years. A liver mass in segment 3 showed increasing size concomitant with increasing alpha-fetoprotein concentration, and a solitary HCC 15 mm in diameter was diagnosed. The tumor was located on the liver surface, abutting the origin of the left hepatic vein. Laparoscopic partial liver resection was performed. The postoperative course was uneventful and the patient was discharged on postoperative day 3. The patient remained disease-free on follow-up after 7 months.Although we had some concerns, such as difficulty managing general anesthesia and easy venous bleeding due to high central venous pressure, laparoscopic partial liver resection was performed with safe exposure of the left hepatic vein. The Fontan procedure (FP) is a palliative surgery for functional single ventricle. The Fontan circulation maintains pulmonary circulation through a high central venous pressure (CVP), leading to chronic congestive liver . Recentlth edition of the classification of the Union for International Cancer Control [4/μL. Alpha-fetoprotein and des-gamma-carboxy prothrombin were elevated to 277.8 ng/mL and 56 mAU/mL, respectively. Type IV collagen 7S was slightly elevated to 8.4 ng/mL, but other markers of liver fibrosis were normal . Negative results were obtained for both hepatitis B virus surface antigen and hepatitis C virus antibody, and the patient had no history of alcohol consumption. The indocyanine green (ICG) retention rate at 15 min was 44%. Ratios of HH 15 (representing blood clearance) and LHL 15 (representing hepatic uptake) on 99mTc-GSA scintigraphy were 0.71 and 0.95, respectively. Child–Pugh classification was A. Echocardiography demonstrated good single right ventricular function and no obstruction in the Fontan circulation. Fractional area change was 42.4%, and common atrioventricular valve regurgitation was mild. Oxygen saturation in room air was 89%.The patient was a 31-year-old man with single right ventricle and congenital asplenia syndrome who had undergone FP at 3 years. Several liver masses were detected at 30 years. One tumor was diagnosed as focal nodular hyperplasia from ultrasound-guided biopsy, and close follow-up was maintained. At 31 years, abdominal dynamic contrast-enhanced computed tomography (CT) revealed another S3 liver mass that had enlarged to 15 mm in diameter. This tumor was diagnosed as HCC based on the appearance of high density from the arterial phase to the portal phase and wash-out in the equilibrium phase . However, since detailed reports of FALD and 99mTc-GSA scintigraphy have not been reported previously, further study is required regarding the assessment of liver function using 99mTc-GSA scintigraphy in cases of FALD. Finally, histopathological examination in the present case demonstrated liver cirrhosis.ICG has commonly been used to evaluate preoperative liver function in Japan, but accurate evaluation is difficult in the presence of factors, such as portosystemic shunt . Howeverhologies . Since t2O in the present case, strict control of airway pressure may also be required to control bleeding from hepatic veins especially in laparoscopic hepatectomy for patients with FALD [Most cases of HCC arising from FALD are reportedly treated non-surgically, with poor liver function reducing the tolerance for surgical resection . Howeverith FALD .The indications for laparoscopic liver resection among patients with FALD thus have to be determined carefully with close communication between the cardiologist and anesthesiologist. Furthermore, we have to keep in mind the timing of conversion to open surgery when performing laparoscopic surgery, not only because of the risk of intraoperative bleeding, but also because changes in vital signs may easily arise in patients with FALD. Liver resection to treat HCC arising from FALD, therefore, requires stricter criteria than conventional liver resection. Such criteria may include preoperative CVP and wedge pressure of the hepatic veins to predict the likelihood of intraoperative bleeding from hepatic veins. To establish such criteria, we should accumulate data on patients with FALD by creating a large-scale, nationwide database.In the present case, a hepatic vein had to be exposed to remove the tumor. However, the wall of the hepatic vein showed fibrous thickening rather than fragility, and no bleeding was observed. Liver congestion is known to lead to fibrous thickening of veins, such as the IVC and hepatic veins . The fibWe have reported a case of HCC abutting the origin of the left hepatic vein after FP, treated successfully with laparoscopic partial liver resection. We hope that the present case will help in the treatment of patients with HCC arising from FALD.


Extra-adrenal myelolipomas are rare, asymptomatic entities, although large tumors may cause local symptoms or hemorrhage. When these lesions occur outside the adrenals in the retroperitoneum, they are radiographically easily confused with both primary and secondary retroperitoneal tumors, which tend to be aggressive. Although myelolipomas are benign and can be managed conservatively, if malignancy is suspected, a surgical procedure is an option. We report a case of a 68-year-old patient with multiple perirenal extra-adrenal myelolipomas. Initial abdominal ultrasound reviled an inhomogeneous mass surrounding the left kidney. Subsequent CT examination of the abdomen showed four separate, extrarenal, well-circumscribed, round-shaped, fat-containing retroperitoneal tumors. Given the significant size of the masses, that compressed major abdominal vessels and the suspicion of liposarcoma, a surgical excision of the lesions was performed. The tumors were easily separated, all surrounding structures were spared, and they were removed completely. Histologically, all masses consisted of hematopoietic and mature fat tissue and the final diagnosis was extra-adrenal myelolipoma. The patient was released from the hospital 7th day after surgery in good condition and at his baseline. Since myelolipomas are, by definition, nonfunctional benign tumors, there was no need for further follow-up. The radiological evaluation and fine needle biopsy are usually sufficient to establish the diagnosis, but in some cases of well-differentiated liposarcoma, the differentiation between myelolipoma and liposarcoma can be challenging. Therefore, considering that myelolipomas and liposarcomas have opposite prognoses, which affects the surgeon's decision on the extent of surgical procedure and further treatment, we also emphasize the importance of intraoperative assessment of the tumor, both by the surgeon and by intraoperative pathology consultation. Myelolipomas are rare tumorous mixtures of adipose and relatively normal trilineage hematopoietic tissue. They usually occur as adrenal tumors (incidence is estimated at lower than 0.1%.), but extra-adrenal myelolipomas have also been described, occurring as head and neck, chest, intra-abdominal, or pelvic masses in up to 15% of all cases  1–4]. P. P4]. PeBoth adrenal and extra-adrenal myelolipomas are typically asymptomatic, although large tumors may cause local symptoms or hemorrhage . Tumors Although adrenal myelolipomas can be managed conservatively, when myelolipomas occur as large multiple retroperitoneal masses, they are radiographically easily confused with both, primary or secondary retroperitoneal tumors. Those could be aggressive neoplasms, such as liposarcoma, requiring different treatment .In this report, we pinpoint the possibility that, although extremely rare, myelolipomas can occur as large, multiple, retroperitoneal, perirenal masses that could be initially mistaken for aggressive neoplasms.A 68-year-old man with lower urinary tract symptoms (LUTS) and well-controlled hypertension was referred to the department of urology. Initial abdominal ultrasound detected an inhomogeneous mass surrounding the left kidney. Complete laboratory exams were normal.Subsequent CT examination of the abdomen reviled four separate, extrarenal, well-circumscribed, round-shaped, and fat-containing retroperitoneal masses . Due to the volume of the masses, the aorta and inferior vena cava were compressed, as well as branches of the superior mesenteric artery. There was no hydronephrosis, and suprarenal glands and other organs appeared normal Figures . Based oGiven the significant size of the masses that compressed the major abdominal vessels and the suspicion of liposarcoma, a decision was made to surgically excise the lesions. The surgical procedure was performed using an open transabdominal approach, under general anesthesia. Tumors were soft and well encased by a thin capsule, intimately attached to the renal adipose capsule, ureter, and abdominal aorta. They were easily separated, all surrounding structures were spared, and the tumors were completely removed . Both suGross pathology revealed encapsulated, well-defined, soft masses measuring 11.5 × 8.5 × 6.0 cm, 15.0 × 10.0 × 6.5 cm, 6.0 × 5.5 × 3.5 cm, and 2.0 × 2.0 × 2.0 cm. Histologically, all the masses consisted of hematopoietic and mature fat tissue . The domThe patient was released from the hospital 7th day after the surgery, in good condition and at his baseline. Since myelolipomas are, by definition, nonfunctional benign tumors, there was no need for any further follow-up.The vast majority of myelolipoma cases have been presented as a single mass. They are asymptomatic, incidentally found on imaging studies, and can be managed conservatively , 8. On uUsually, extra-adrenal myelolipoma can be diagnosed by CT or MRI scans with great certainty , 7, and Open surgery is a gold standard for the treatment of large myelolipomas. On the other hand, a transperitoneal or retroperitoneal approach with minimally invasive surgery is possible, as shown by Arezzo et al. . FurtherCurrently, there are no consensus recommendations for postoperative surveillance of myelolipoma . ClassicAlthough myelolipomas are rare, and especially multiple extra-adrenal retroperitoneal types, we would like to remind the surgeons, radiologists, and pathologists to be aware of these tumors. The radiological evaluation supported by histological examination upon the fine needle biopsy is usually sufficient to establish the diagnosis, but in some cases of well-differentiated liposarcoma, telling the difference between myelolipoma and liposarcoma can be challenging. Therefore, considering that myelolipomas and liposarcomas have a diametrically opposite prognosis, which then drives the surgeon's decision on the treatment, we also emphasize the importance of intraoperative assessment of the tumor, both by the surgeon as well as by intraoperative pathological evaluation.


A 52-year-old immunocompetent man presented with frontal headaches. Physical examination showed a painful frontal hump (A). Computed tomography (CT) and magnetic resonance imaging (MRI) scans revealed a giant bifrontal extra-axial tumor . He benefited from total removal of the tumor. Pathological examination of the resected mass led to the diagnosis of anaplastic large B-cell lymphoma (cell immunopositivity for CD30 and anaplastic lymphoma kinase) (D). He was transferred to the oncology unit for chemo and radiotherapy with good clinical evolution. Primary dural lymphoma (PDL) is extremely rare, accounting for 7% of all primary central nervous system lymphomas. PDL usually involves sites that are rich in meningothelial cells, and results in a localized mass or plaque-like thickening of the dura that radiologically resembles a number of other diseases, such as meningioma or subdural hematomas. Although there is as yet no optimal treatment for PDL, surgical excision followed by radiotherapy and chemotherapy are recommended.


A male 28-year-old patient complained of a dorsal mass that has been increasing in size in the last six months. The mass was painful, soft, no mobile, and no neurological symptoms or signs were documented. A vascular-type tumor was suspected and endovascular followed by open surgical resection was indicated.Histopathological revealed a rare case of an adult with a primary extranodal polymorphous hemangioendothelioma. Total resection was confirmed by tumor-free resection margin. The postoperative course was uneventful.Polymorph hemangioendothelioma is a rare vascular tumor. Preoperative endovascular embolization is recommended given the high vascular features of the lesion and, therefore, the high rate of bleeding during surgery, to achieve complete resection. Hemangioendothelioma is a rare vascular tumor that was first described in 1982 [The first three cases of polymorphous hemangioendothelioma (PH) were described in 1992 in an analysis of 39 cases of primary vascular lymph node tumors . A revieGiven the small number of cases found, the natural history of the disease has not been elucidated. However, the potential for PH to metastasize and recur has been described ,6,8 and,A 28-year-old male patient presented to the outpatient clinic due to a “lump” in his dorsal back that had been increasing in size in the previous six months. He did not report any weight loss, fever, or neurologic deficit. The patient had been referred to a different hospital where an open biopsy procedure was performed two months earlier but due to significant blood loss, the procedure had to be suspended. The histopathology result described a lesion compatible with hemangioma. Since then, the patient had noted a significant size increase. The patient had no other medical conditions and no medication use. Physical examination showed a dorsal 10 cm mass, non-mobile, slightly painful, with no erythema or secretions associated. The rest of the examination was unremarkable.A thoracic spine contrast-enhancing magnetic resonance imaging (MRI), revealed a dorsal mass at T8 level with contrast enhancement, compromising only soft tissues. The mass did not involve the vertebrae or medullary canal. No lymph node enhancement was documented. Given the blood loss history during the previous surgical procedure, spinal arteriography was indicated to assess mass arterial supply. The spinal arteriography Figure  was perfOne day after embolization, the patient was taken to open surgical resection. Dissection through the tumor plane was performed, the vascular supply from T10 and T11 radiculomedullary branches was sparse due to previous endovascular embolization. No significant bleeding occurred and complete resection of the lesion was achieved Figure , which wHemangiothelioma is a borderline vascular tumor between benign hemangioma and malignant sarcoma. Seven different subtypes of properly hemangioendothelioma have been described Table .The PH natural history, rate of recurrence and potential to metastasize is not well known given the limited available reported cases in the literature. Likewise, the first-line treatment has not been standardized, but total resection is recommended. Additionally, immunotherapy and radiotherapy has been proposed as adjuvant treatment ,2.Given the vascular etiology of the PH, operative bleeding can be of concern and should be addressed. This case was approached with preoperative arteriography that revealed arterial supply from radicular T10 left artery and, in the absence of Adamkiewicz artery, embolization was indicated and could be performed without complication. Afterwards, open surgical resection was performed with low operative bleeding and complete resection with tumor-free edges was obtained. This is the first case of extranodal, primary PH treated with endovascular coiling followed by open resection, and showed exceptional results.PH is a rare vascular tumor. The classification and natural history depend on localization . The focal, extranodal primary polymorph hemangioendothelioma could be treated with a combined neurosurgical approach consisting of endovascular coil-embolization followed by resection surgery, which can enhance total resection, decreasing the risk of surgical complications as incomplete resection or bleeding.