Spirometra erinaceieuropaei sparganosis presenting with two nonmigratory nodules in close proximity to each other on the right thigh of a Thai woman living in Switzerland.Human sparganosis is a cestode infection which is neglected as a differential diagnosis outside endemic countries. Diagnosis and therapy may be challenging depending on the clinical presentation and anatomic localization. The disease manifests predominantly as subcutaneous nodule(s) or intracranial mass lesion(s). Infection is primarily acquired by ingesting raw or undercooked amphibian or reptile flesh or by drinking water containing copepods. We report an unusual case of subcutaneous  In June 2018, a 55-year-old Thai woman living for the last 25 years in Switzerland presented with a history of two subcutaneous, painless, nonitching nodules to a dermatologist in Switzerland. The nodules had been present for 4 years, and the patient reported neither growth nor change of morphology over time. On palpation, two firm subcutaneous nodules of coarse consistency, both measuring approximately 4 cm in diameter, located on the right upper thigh in close proximity to each other, were identified. Neither signs of inflammation nor local lymphadenopathy was present, and the physical examination was otherwise unremarkable. Basic laboratory investigations  were all within normal limits. Because the clinical presentation suggested a benign soft tissue process, imaging was dispensed under the assumption that definite diagnosis would be established by surgical excision and histological examination. Hence, the patient was referred to a plastic surgeon. On incision of the first nodule, a whitish, thin, flattened, worm-like object (∼0.3 × 15 cm) was pulled out. On incision of the second nodule, a morphologically similar structure was removed . (Note: 1 A 398–base pair-long sequence of the COX1 was determined (GenBank accession number MK910864) showing 100%, 99.25%, and 90.20% sequence homology to Spirometra erinaceieuropaei (no single nucleotide polymorphisms [SNPs]), Spirometra decipiens (3 SNPs), and Sparganum proliferum (> 35 SNPs), respectively  were sent to the National Reference Center for Human Parasitic Diseases at the Swiss Tropical and Public Health Institute in Basel. Macroscopic and microscopic investigation of the sample stored in normal saline solution showed an amorphous structure exhibiting slow, undulating movements. An anterior end was identified by finding an oral opening of a helminth, whereas the posterior end was disrupted. A part of the sample was embedded in paraffin and used for histological investigation, which revealed typical characteristics of spargana: a folded cuticula with microvilli and calcareous corpuscles . For speectively . Thus, tSpirometra spp. In the Old World, S. erinaceieuropaei causes most infections, whereas Spirometra mansonoides is mostly prevalent in the New World.3Sparganosis is a rare zoonotic cestode infection caused by the second-stage larva of various species of the pseudophyllidean tapeworm 4Approximately 1,600 human cases have been reported worldwide since the first case description in 1882 in Xiamen, Fujian Province, China. More than 80% of all cases have been reported from China, followed by Korea, Thailand, and the United States.Diphyllobothrium spp. Carnivorous mammals serve as definite hosts, harboring the adult worm in the intestine and shedding eggs in their feces. When eggs get into freshwater, they release coracidia. Coracidia are ingested by copepods, the first intermediate host, and develop into procercoid larvae (first-stage larvae) within 3–11 days.5 When the copepod is ingested by fish, reptiles, or amphibians, the second intermediate hosts, procercoid larvae develop into plerocercoid larvae , lodging in various body tissues. When the second intermediate host is ingested by the definite host, plerocercoid larvae develop into adult worms in its intestine. In addition, several predator species may serve as paratenic hosts , may be infiltrated by the larvae and manifest as nodular mass lesions. A review from Korea revealed the subcutaneous tissue and the CNS as the most frequently infiltrated anatomic sites, accounting for 49.9% and 36.2% of cases, respectively.8The incubation period in humans varies from several days up to years.10 In general, reports on multiple lesions, like in our case, are rare. Case reports like that of a Korean woman with a history of eating snakes 40 years before developing multiple masses on different body sites  are exceptional.11The patient described in our case frequently travels to her home country Thailand, where at least 62 cases have been reported in the past 60 years. In most cases, sparganosis presents as a single subcutaneous lesion. Of note, ocular involvement due to applying frog flesh poultices in traditional medicine was described in one-third of cases in Thailand up to the 1990s. Since then, the number of such cases has decreased, probably because of increasing disappearance of this practice.Spirometra proliferans, where multiple masses may occur because of the worm’s ability of branching and budding, resulting in a more serious clinical outcome.4A differential diagnosis to be kept in mind for prognostic reasons would be infection with Multiple infections may also occur in case of local inoculation of the larvae due to applying poultices made out of reptile or amphibian flesh. However, our patient denied having used this form of traditional medicine.8 Wiwanitkit identified the history of drinking “impure” water as the main risk factor for human sparganosis in Thailand.12The patient also denied ingesting raw or undercooked reptiles or amphibia. However, she reported drinking filtered tap water at her hometown in Thailand. Thus, the most likely route of infection remains drinking copepod-contaminated water, as filtration might have been insufficient. A review highlighted ingestion of snakes or frogs observed in 63.4% of the presented cases, whereas drinking unfiltered water accounted for 16.9% as the suspected infection route.13 who found eosinophilia in only 30% of sparganosis cases.Blood eosinophilia, usually suggestive of a tissue-invasive helminth infection, was absent in our patient. This is in line with observations by Zhang et al.,14The initial differential diagnoses in our case were limited to sebaceous cysts, fibroma, and lipoma because typical characteristics of tissue-invasive helminth infections, such as creeping eruption/migratory swelling or blood eosinophilia, were absent. When such characteristics are present, differential diagnoses primarily depend on epidemiology and exposure. In the case of nonmigrating lesions, dirofilariasis or soft tissue cysticercosis could also be considered especially because blood eosinophilia is absent in most of these cases too.S. erinaceieuropaei in our case is in line with two case series from Thailand where genotyping revealed S. erinaceieuropaei in all 12 investigated cases.10 The diagnosis is usually made by histological identification followed by molecular confirmation of the parasite, after surgical excision. When surgery is not feasible, serology and imaging remain important diagnostic tools. Although the use of highly sensitive serological assays has been described in publications from endemic regions, serological testing remains largely unavailable in most nonendemic settings.15 In the case of subcutaneous sparganosis, a band-like structure may be seen on magnetic resonance imaging. Ultrasonography may show a coiled linear hypoechoic body in a clear oval mass.16Identification of S. proliferum infections, which may show continued growth in the case of incomplete removal. Histological identification of Sparganum spp. is based on the finding of a characteristic deep-folded tegument and calcareous corpuscles which are unique to cestode tissue.17 However, in this developmental stage of the parasite, a definitive species diagnosis can only be achieved by molecular methods. Up to now, no standards for molecular identification are defined. Most commonly, the mitochondrial COX1 gene is amplified and sequenced. In this context, it needs to be noted that amplification and sequencing is based on intact DNA and that formalin (which causes severe DNA damage) should, therefore, be avoided for sample preservation in such cases.Species identification is desirable so as not to miss 18Treatment of sparganosis is primarily based on surgical removal of the larva(e). No medical treatment standard has been defined for patients suffering from masses not suitable for surgical resection. However, high-dose praziquantel (repeated cycles of 50–75 mg/kg body weight/day for 10 days) has been reported to be effective in inoperable cases of cerebral sparganosis.Spirometra spp. are rare, usually not considered outside endemic regions, and as in the case presented, diagnosis is only made after surgical removal of the parasite. There is a lack of commercially available serological tests, and species identification requires sequencing.In summary, human infections with Supplemental materials


A giant congenital cervical teratoma is often highly vascularized; thus, in addition to a life-threatening airway occlusion at birth it comprises a high risk for significant and lethal blood loss during resection. In the case presented, an endovascular embolization of the carotid artery that supplied a giant congenital cervical teratoma was done as part of a three-stage treatment soon after birth and contributed to an overall good outcome. Embolization in cases of cervical teratomas was not described previously.ex utero intrapartum treatment procedure after extensive preoperative planning and followed by tracheostomy, (2) endovascular embolization of the carotid artery that supplied the tumor in order to decrease blood loss during resection, and (3) complete surgical resection. The parents were involved in all the ethical and medical decisions, starting just after the cervical mass was diagnosed prenatally.We present a case of a preterm newborn from a Sephardic jewish origin with a giant, highly vascularized, congenital cervical teratoma that was managed successfully in three stages: (1) delivery by an The management of giant congenital cervical teratoma is often challenging from both a medical and ethical prospective. Meticulous perinatal planning and parents’ involvement is crucial. Endovascular embolization of the tumor feeding vessels can significantly improve the resection outcome and overall prognosis. We presAn otherwise healthy 33-year-old woman, gravida 3, para 2, from a Sephardic Jewish origin, was initially referred to our institution at 30.6 weeks of gestation due to a large neck mass found on prenatal ultrasonography (US). Her previous two pregnancies were uncomplicated. The fetal sonogram showed a 10 by 8 cm mass on the right side of the neck, which was not present in detailed scans taken at 14 and 22 weeks. The mass was composed of a cystic portion and a solid portion containing blood vessels and was growing rapidly in subsequent ultrasound studies. A significant polyhydramnios with amniotic fluid index (AFI) of 50 suggested an upper gastrointestinal obstruction and a highly possible airway obstruction as well. Findings were confirmed by fetal magnetic resonance imaging (MRI). In anticipation of the difficulty in establishing a secured airway at birth and the potential complicated resection of the giant tumor after birth, the mother was referred to our hospital for consultation.The parents were in consultation with the maternal fetal team, neonatologist, anesthesiologist, pediatric surgeon, and otolaryngologist. The parents were presented with a guarded prognosis but insisted that the pregnancy continue with maximal efforts during delivery and during the neonatal period.Therefore, a planned EXIT procedure, which provides the best chance to establish a patent airway, was offered to our patient, presenting the risks . SpecifiKnowing the risk of an unplanned preterm delivery due to polyhydramnios and uterine contractions, we scheduled our patient for a planned cesarean delivery at 34 weeks organizing and preparing a multidisciplinary team ready to perform the EXIT procedure.A multidisciplinary team including obstetricians, anesthesiologists, neonatologists, otolaryngologists, pediatric surgeons, pulmonologists, cardiologists, and nursing staff participated. A combined epidural and general anesthesia was planned. Our patient’s blood pressure was monitored continuously to detect and treat a possible event of maternal hypotension in order to maintain good fetal perfusion. After epidural catheter placement while lying on her left side, the parturient returned to lie on her back. Immediately, a severe hypotension (65/30 mmHg) with tachycardia (150 beats/minute) appeared. We related this complication to the polyhydramnios causing a significant decrease in the vena cava flow. After left uterine displacement and bolus of phenylephrine, her blood pressure and heart rate returned to normal. General anesthesia with rapid sequence induction was induced with succinylcholine (100 mg) and propofol (150 mg). During the EXIT procedure an appropriate uterine relaxation was maintained to prevent placenta expulsion. We used a high dose of inhaled anesthetics and minimal nitroglycerine intravenous drip. No bleeding occurred during the procedure.in situ to avoid placental separation. The amniotic fluid was slowly drained to avoid an abrupt drainage of the fluid and an early separation of the placenta.A classical uterine incision was made and only the fetal head and upper chest with the cervical mass were delivered through the uterine incision. The rest of the body and the cord were left The multidisciplinary team had planned and rehearsed the following escalating step-by-step scenarios for establishing a secured airway: (1) direct laryngoscopy and intubation attempt by a pediatric anesthesiologist with the aid of a neonatologist, (2) rigid bronchoscopy by an otolaryngologist and possible aid of flexible bronchoscopy by a pediatric pulmonologist, (3) if laryngoscopy and bronchoscopy failed, a tracheostomy was planned by an otolaryngology team. As the tumor was highly vascularized, any debulking procedure was impractical and would have imminently put both the mother and newborn at the risk of death. Analgesia for the newborn using intramuscular fentanyl was prepared in advance.2 of 71 mmHg and lactate of 9.5 mmol/L. Immediately after delivery, a computed tomography (CT) scan was performed under general anesthesia that demonstrated that a tracheostomy could be performed without interfering with the tumor. During the tracheostomy, a biopsy was taken from the tumor that demonstrated both mature and immature teratoma , the tumor was dissected including a section of the lacerated skin. Because the main blood supply of the tumor was embolized, the surgery was performed from its distal part along our patient’s mandible towards the proximal part at the junction of her neck and thorax. The tumor impinged into her pharynx, and her larynx and epiglottis were identified and preserved. Her mandible and neck muscles were preserved; however, her vagal nerve was surrounded by the tumor and was sacrificed with the carotid artery. The tumor weighted 800 grams and the overall blood loss was 300 ml. After tumor resection and closure of the skin (Fig. Less than 24 hours after the embolizationGradually, after the tumor resection, our patient was weaned from mechanical ventilation. She was fed initially using an orogastric tube and gradually learned to feed orally. After the resection, a right vocal cord paralysis was observed using a flexible fiberoptic laryngoscopy; therefore, we decided to leave the tracheal tube in place. We attributed this finding to an injury of the recurrent laryngeal nerve during the resection. Another neurological sequela was an abduction weakness of her right shoulder which slowly recovered. Neck teratomas can arise from and completely replace the thyroid tissue . ThyroidCervical teratomas can acquire gigantic dimensions in late gestation. The compression over the esophagus causes polyhydramnios and the associated compression over the trachea can cause airway obstruction at birth.Severe polyhydramnion may lead to preterm delivery, thus complicating the upper airway obstruction with immature lungs and respiratory drive. Planning secure resuscitation at birth needs to take into account the availability of multidisciplinary teams and the issue of prematurity; thus finding the best timing for intervention.EXIT is a surgical delivery procedure used to deliver fetuses who have airway compression. Failure to achieve airway under controlled and safe conditions and in a reasonable time might result in asphyxia.in utero diagnosis, and following thorough assessment and consultations, our team felt that the risk for the mother during the EXIT procedure and the risks and possible sequelae for the infant during and after the different procedures after birth were significant enough that offering no treatment was ethically reasonable. The parents, fully aware of all options and risks, decided on maximal care to their daughter.Giant congenital cervical teratomas are challenging lesions. After making the In the case of our patient, once stabilized with tracheostomy after EXIT, we found the tumor to have a highly abundant blood supply. Although pre-excision embolization was described sporadically in cases of sacral teratoma , 8, we fThe clinical challenges reported in this case highlight the importance of multidisciplinary teams in the management of congenital cervical tumors. This case also emphasizes the ethical issues and the need for an open discussion with the parents in a similar life-threatening prenatal diagnosis.


An acute scrotal hematoma, secondary to a spontaneous rupture of a varicocele is still a rare presentation in daily practice. However, multiple case reports have been reported. Sudden increase in abdominal pressure, resulting to an increased venous pressure can lead to a rupture of the varicocele. Literature search shows that due to uncertainty of the diagnosis, explorative surgery is often performed, sometimes resulting in unnecessary orchiectomies. The objective of this study was to determine classical clinical presentation of patients with a spontaneous rupture of a varicocele, determine the diagnostic procedure, and give an insight in the follow-up.We present a case of a 24-year old male with acute scrotal swelling after sneezing. Subsequently, we carried out a systematic literature search to identify all eligible studies to determine classic clinical presentation of spontaneous ruptures of a varicocele.The literature search shows that clinical presentation of idiopathic spontaneous scrotal hematomas is similar to testicular torsion, epididymo-orchitis, malignancy, or (incarcerated) inguinal hernia making differential diagnosis difficult. Especially when there has been increased abdominal pressure or strenuous activity preceding the symptoms, and the swelling is left sided, it should be included in the differential diagnosis for patient with acute inguinoscrotal swelling. Colour Doppler-Ultrasonography is recommended to distinguish between other causes of acute scrotum. The hematoma is usual self-limiting, justifying conservative treatment. Early surgical intervention is indicated with signs of ischaemia due to obstruction, infection of the hematoma, or uncertain diagnosis . However, physicians should be cautious with direct exploration, as it led to unnecessary orchiectomy in 25% of patients. The hematoma can increase in size up to 3 months post-event, and it can take up to 15 months to completely resolve. A varicocele is present in about 15% of healthy males . A spontA literature search on this topic during treatment of this patients resulted in the finding that due to uncertainty, explorative surgery is often performed, sometimes resulting in unnecessary orchiectomies. The aim of this study was to determine classical clinical presentation of patients with a spontaneous rupture of a varicocele, determine the diagnostic procedure and give an insight in the follow-up.A 24-year old male was seen at the Emergency Department of our hospital with acute scrotal swelling on the left side, which started 5 days earlier. The symptoms started during a trip to Japan, where the patient had multiple severe sneezes while walking outside. On examination, he had a large swelling of the left hemiscrotum. Except for a left sided varicocele Fig. , which wThree months post-event, the hematoma even further increased in size to 50x37x30mm. Eventually, the patient underwent a microscopic inguinal varicocelectomy. After, the hematoma showed signs of reabsorption, decreasing in size to 38x24x21mm 4 months; 20x16x11mm 6 months; and to no residual hematoma eventually, 15 months post-event. The left testis itself did not differ in size at all follow-up points.An acute scrotal hematoma, secondary to a spontaneous rupture of a varicocele is still a very rare presentation in daily practice. However, multiple case reports have been reported.We carried out a systematic literature search for PubMed, EMBASE, and Cochrane Library, using Medical Subject Headings indexes, keyword searchers and publication types until May 2017. Additionally, the reference lists of included articles/case reports were also examined for potential studies. There was no language restriction, as long as all relevant info was present (in the abstract), and the abstract was in English. The following keywords were used in the search: varicocele rupture, or spermatic cord hematoma, or hematocele spermatic cord. The terms were separately searched or connected by the Boolean operators “AND” and “OR”. We identified in our search 31 case reports. Differentiation could be made based on etiology into three major causes, i.e. idiopathic, spontaneous hematomas (18×) –26, as a. showed that patients with a left-sided varicocele develop an increased venous pressure during a Valsalva manoeuvre [Idiopathic, spontaneous hematomas are thought to be the result of sudden increase of abdominal pressure transmitting to a varicocele. It’s arbitrary whether abdominal trauma can lead to increased abdominal pressure, resulting in a ruptured varicocele . A studyanoeuvre . Variousanoeuvre .Average age of presentation was 31 ±18) years, with the overall majority of males in their 20s at presentation. This makes distinction between possible testicular torsion, or malignancy difficult, since both affect mostly males between 15 and 35 . Accurat years, wReported clinical presentation of patients with a spontaneous ruptured varicocele is acute pain (83%), and acute  scrotal swelling (100%), which could also be difficult to distinguish from testicular torsion, appendix testis torsion, or malignancy. Important to note is that patients with testicular cancer commonly present with a painless mass. Just 10 % of patients present with acute symptoms such as pain , so whenDiagnosing a cause of acute scrotal pain and swelling can be difficult, and as stated the differential diagnosis should include conditions such as testicular torsion, malignancy, epididymo-orchitis, or (incarcerated) inguinal hernia , 14.Blood analyses and coagulation tests could be performed to exclude any bleeding disorders. In one case, symptoms mimicked an epididymo-orchitis, due to leucocytosis . No ultrCUD is the imaging modality of choice in evaluating patients who present with acute scrotal pain , especiaImaging of the scrotum was performed in all but four cases , 12, 16,Surgical exploration is indicated when diagnosis is uncertain, or when a testicular torsion or ischaemia cannot be excluded, and orchiectomy can be performed when malignancy is highly expected, . However, physicians should be cautious for unnecessary orchiectomy. When performing exploration, an inguinal approach is recommended, if malignancy is possible, to avoid scrotal violation. Additional arguments for exploration are possible future complications, i.e. testicular ischaemia due to obstruction of the hematoma on the funiculus , possiblOverall, exploration was performed in ten patients 56%), indicating the lack of knowledge about idiopathic spontaneous hematomas of the scrotum. Direct exploration at presentation was performed in eight patients 44%). Reasons of direct exploration were uncertain diagnosis 63%) 4%. Reaso, 16, 18,% 4%. Rea%, indicaPresent varicocele is usually diagnosed with imaging or surgical exploration after the forming of the hematoma. Besides the case report of Gordon et al. 1993 , this isIn general, the scrotal hematoma is considered self-limiting and should be treated conservatively. Symptoms can be treated with NSAID’s, and RICE .In our patient, the hematoma increased in size up to 3 months post-event. Only after microscopic inguinal varicocelectomy the size of the hematoma started to decrease. It is unsure whether this is a result of the treatment or time. After 15 months, the hematoma was dissolved completely. It is important to inform the patient of the possible duration of completely reabsorption to avoid anxiety and unnecessary follow-up diagnostics.Avoiding increased abdominal pressure for patients with known varicocele, to avoid spontaneous ruptures does not seem indicated. The scarcity of case reports, as a complication of a condition present in about 15% of males, shows its rarity. Recurrent hematomas have never been described.An acute scrotal hematoma secondary to a spontaneous varicocele rupture is a rare phenomenon. A literature search on case reports shows that clinical presentation is often similar to testicular torsion, appendix testis torsion, or malignancy, making differential diagnosis difficult. Especially when there has been increased abdominal pressure or strenuous activity preceding the symptoms, and the swelling is left sided, it should be included in the differential diagnosis for patient with acute inguinoscrotal swelling. Colour Doppler-Ultrasonography is the golden standard to distinguish between other causes of acute scrotum. Early surgical intervention is justified with signs of ischaemia due to obstruction, infection of the hematoma, or uncertain diagnosis . However, physicians should be cautious whereas direct exploration was performed in almost half the case reports, which led to unnecessary orchiectomy in 25% of patients. Otherwise, the hematoma is self-limiting, and conservative treatment is recommended. The hematoma can increase in size up to 3 months post-event, and it can take up to 15 months for the hematoma to completely resolve.


This report describes a huge chondromyxoid fibroma (CMF) that developed in the right iliac crest and wing. The tumour is rare, perhaps the rarest of all bone tumours, and its occurrence in the iliac crest and wing of a 63-year-old male is extraordinarily uncommon. The patient complained of gradual onset of right groin pain over a period of more than 2 years and low back pain and tender swelling of the right gluteal region over a period of another 1 year. Conventional radiography of the lumbar spine and pelvis revealed a large osteolytic lesion of the right iliac crest and wing associated with mild levoscoliosis. MRI of the pelvis revealed a huge well-defined lesion arising from the right iliac crest and wing and extending to the right paraspinal region, false pelvis and right gluteal region and displacing rather than invading the surrounding structures. The patient underwent surgery, and the mass was totally removed. The clinical manifestations, imaging findings and surgical treatment of the lesion are discussed. Its documented incidence is less than 1% of all primary bone tumours  with males and females being equally affected. It has a potential for regional enlargement towards the local tissues. It consists of immature myxoid mesenchymal tissue with features of primitive cartilaginous differentiation. Patients most commonly affected are in their second or third decade of life. An additional peak of incidence is observed between 50 and 70 years of age.T2 weighted images, and the diffuse low signal on T1 weighted images. CMF of flat bones of the pelvis could reach a huge size, and it could extend to the paraspinal region, the pelvis and the gluteal region, displacing and not invading the surrounding structures.The imaging features of CMF in the radiological literature mostly apply to lesions arising from the long bones. The current report aims to describe the imaging features and behaviour of CMF arising from the flat bones of the pelvis and to differentiate it from more aggressive lesions such as chondrosarcoma. The MR criteria of CMF are the peripheral low to the intermediate signal band and the central hyperintense signal on 2CMF with atypical radiographic findings may mimic more common and more aggressive tumours. The differential diagnosis of CMF includes a variety of bone lesions. CMF lesions at unusual sites or age groups could lead to misinterpretation and unnecessary investigations.A 63-year-old male presented first with right groin pain and back pain over a period of more than 2 years and received analgesics, which did not help much. A year later, he started to feel pain and swelling in the right gluteal region.Conventional radiography of the lumbar spine showed mild left convex scoliosis and a partially elicited right iliac wing osteolytic lesion .Conventional radiography of the pelvis revealed a large osteolytic lesion involving the right iliac crest and wing with sclerotic margins and no obvious dominant gross matrix calcifications .T1 homogenous hypointense and T2 heterogeneous hyperintense signal with internal dominant fluid-equivalent signal and peripheral nodular hypointense margin. The mass is saddle shaped, overhanging the right iliac crest with a pelvic  limb displacing and attenuating the right iliopsoas muscle without invasion of the iliac vessels and gluteal  limb extending into the gluteal region between the gluteal medius and minimus muscles. No evidence of invasion or encasement of the surrounding structures or adjacent iliac vessels was observed, and there was no defined regional pelvic lymphadenopathy. The whole lesion was surgically excised. There was no defined invasion of the surrounding adjacent soft tissues of the pelvis or the right gluteal region (MR examination of the pelvis elicited a huge (about 176 × 129 × 94 mm) mass arising from the superior aspect of the right iliac wing, which is generally l region –7.CMF is a rare benign bone tumour accounting for about less than 0.5% of all bone tumours. It is a very slow-growing tumour and usually presents with a large mass. The matrix is predominantly cartilaginous with varying chondroid, fibrous and myxoid elements.3All the bones could be affected, however; the most common sites of involvement include the metaphysis of long bones. Involvement of lower limbs is 5-fold more common than that of the upper limbs. Involvement of the small bones of the feet is 5-fold greater than that of the hands.4CMF has a predilection to occur in the knee region, especially in the proximal tibial metaphysis. Only five cases of chondromyxoid fibroma of the pelvis have been reported.Pelvic bones are a rare location for CMF. Iliac wings are an unusual site for this rare benign bone tumour of cartilaginous origin. A wide variety of benign and malignantlesions can arise from the pelvic bones. The differentiation between such lesions mainly relies on the patient’s age, gender, the number of lesions, type of matrix and the presence or absence of invasion of the surrounding structures and regional or remote lymphadenopathy. Conventional radiography and CT are necessary for matrix characterization, particularly, detection of mineralization. MRI is helpful for further characterization and radiological differential diagnosis.5Small CMFs are usually asymptomatic. Large lesions might cause local pain, swelling or distortion of bone.6The radiological appearance of CMF is not specific and may often mimic more common bone lesions, particularly when it arises from an unusual site such as the iliac wing. These radiological findings are variable, depending on the anatomical site of the lesion. CMF could be considered in the differential diagnosis in cases with well-defined multilocular lesions with sclerotic borders and absent periosteal reaction. An expansile ovoid lesion with a radiolucent centre is the usual radiographic pattern. Well-defined sclerotic margins, internal septations and a bulging thinned overlying cortex are frequent CMF findings.3The conventional radiographic features of chondromyxoid fibroma are expansile, lytic, eccentric and well demarcated. Calcification might exist with a variable incidence ranging from 11 to 14%.7Internal septations might exist. The presence, pattern and distribution of matrix calcifications are better identified and characterized by CT, not conventional radiography.4Microscopically, there is a higher incidence of calcifications up to 34%, much more than that detected on conventional radiography or CT. Calcifications are more prevalent in older patients and in flat bones.T1 weighted images. Peripheral low to intermediate margin and central heterogeneous bubbly high signal intensity on T2 weighted and short tau inversion-recovery images with predominant fluid-equivalent signal are seen.8The tumour typically demonstrates heterogeneous low signal intensity on 9Heterogeneous matrix enhancement achieved post-contrast injection is typically more marked at the vascular borders of the tumour. Heterogeneity is due to the existence of cystic and/or haemorrhagic components of the lesion.10MR features of the CMF still are nonspecific, yet considered the mainstay for preoperative planning.A single imaging modality alone is insufficient for making a diagnosis of CMF. Combination of imaging modalities such as conventional radiography, MR and/or CT is recommended to characterize the lesion and narrow the spectrum of differential diagnosis.Most criteria of CMF in the imaging literature are applicable to the more common sites of origin of long bones about the knee region. Few cases in the imaging literature described the CMF of flat bones, namely the iliac wings.11The imaging differential diagnosis of CMF should consider the site of origin of the lesion and includes a variety of bone lesions such as cartilaginous tumours , osteogenic tumours (osteoid osteoma and osteoblastoma), fibrous tumours (non-ossifying fibroma), non-neoplastic reactive lesions  and other benign lesions . Malignant tumours  must also be considered in certain cases.12An aneurysmal bone cyst inside a CMF is rare but possible.13Malignant transformation of CMF to chondrosarcoma was seen in several cases, yet none of those cases were sufficiently documented.14Some cases of CMF showed partial features of chondrosarcoma without metastasis. The hypothesis of primary malignant CMF was rejected.15The first-year postoperative recurrence rate could reach up to 11%. Incomplete surgical excision carries the risk of higher recurrence rates (up to 80%). Radiotherapy could lead to malignant transformation.4Total excision with safety (tumour-free) margins is the treatment of choice for CMF. Intralesional curettage is associated with high (up to 25%) recurrence rates. Despite the benign nature of CMF, its local aggressiveness should not be underestimated.T1 weighted images and heterogeneous, peripheral low to intermediate and central high signal intensity on T2 weighted images.CMF shows indistinctive imaging patterns in most cases. Sclerotic rim and sometimes bubbly-appearing lesions appear on conventional radiography. CT scans might reveal calcifications within the tumour that is not visible on conventional radiographs. When occurring in unusual locations or in older patients, as in the current case, careful differentiation from more serious and aggressive tumours such as chondrosarcoma is a must. The tumour typically demonstrates diffuse low signal intensity on The presented case is unusual with regard to patient’s age, site of involvement, overall lesion size and extensions. The mass overhangs the iliac crest and extensively extends to the paraspinal region, pelvis and gluteal region with displacement and compression rather than the invasion of the surrounding structures.Imaging evaluation and analysis, particularly MR, is generally useful in distinguishing CMF from other possible entities. Multimodality imaging workup is probably required to narrow the scope of differential diagnosis of CMF to overcome the indistinctive imaging features of the tumour on each single modality and to achieve an accurate estimation of the margins and soft tissue extensions and relations of the lesion. Imaging is crucial to differentiate CMF from more aggressive lesions and to plan for surgery.CMF is a rare, very slowly growing tumour, and usually involves patients in the second and third decades of life, with a predilection to the metaphysis of long bones.When occurring in unusual locations or in older patients, careful differentiation from more serious and aggressive tumours such as chondrosarcoma is crucial.T2 weighted images. T1 weighted images showed hypointense to intermediate signal intensity throughout the lesion.The helpful MR features of CMF are the peripheral intermediate signal band and the central hyperintense signal on Despite the benign nature of CMF, its local aggressiveness should not underestimated.Multimodality imaging workup is probably needed to narrow the scope of differential diagnosis, differentiate CMF from more aggressive lesions and meet proper management planning.Written informed consent for the case to be published  was obtained from the patient(s) for publication of this case report, including accompanying images.


Kaposiform hemangioendothelioma (KHE) is a rare and locally aggressive vascular tumor with histological features resembling Kaposi sarcoma and capillary hemangioma mainly occurring in children and adolescents. Approximately 200 cases have been reported since its original description in 1993, with the vast majority presenting at an early age as raised ill-defined lesions with a red-blue hue mainly involving the skin and soft tissues in the extremities. Cases in adults remain extremely rare. Herein, we describe the case of a 29 year-old man who presented with progressive abdominal pain for 4 months and signs of obstipation found to be consistent with small bowel volvulus. The patient underwent exploratory laparotomy and resection of 55 cm of necrotic small bowel followed by enteroenterostomy and anastomosis. Microscopic examination revealed KHE involving small intestinal mesentery, muscularis propria, and submucosa. His recovery was uneventful and he was discharged after stabilization, opting to manage him expectantly with abdominopelvic imaging and to monitor for development of Kasabach-Merritt phenomenon. To our knowledge, this represents the first reported case of this entity presenting as intestinal obstruction in an adult for which we also present a review of the existing literature and possible treatment options. Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm with histological features resembling Kaposi sarcoma and capillary hemangioma that mainly occurs in children and adolescents. Reports in adults are exceedingly rare. An extensive search in PubMed using the keywords “Kaposiform” and “hemangioendothelioma” failed to reveal any reports of KHE presenting as intestinal obstruction outside of early childhood. To the extent of our knowledge, this represents the first reported case of this entity presenting as intestinal obstruction in an adult, an unusual presentation in an atypical patient population.A previously healthy 29-year-old obese man of Hispanic descent with no significant past medical or surgical history presented with complaints of progressive epigastric and periumbilical abdominal pain of 4-month duration, with an acute exacerbation 2 days prior to his initial visit in our institution. He also experienced associated nausea and emesis, fevers, and chills with obstipation and no passage of flatus. On admission, he was tachycardic and febrile. On physical examination, he had a distended abdomen, which was also diffusely tender to palpation. There were audible borborygmi. History and physical exam were concerning for small bowel obstruction.Routine laboratory investigations were unremarkable, except for mild hyponatremia and hypochloremia. A computerized tomography (CT) scan of the abdomen and pelvis with contrast revealed multiple dilated loops of small bowel in the midline upper abdomen with thickening of the intestinal wall, mucosal hyperenhancement, and fecalization of small bowel loops which appeared to loop on themselves, suggesting small bowel volvulus. Imaging further revealed adjacent inflammatory changes in the mesentery characterized as fat stranding, multiple enlarged mesenteric lymph nodes, questionable pneumatosis intestinalis, and free fluid in the pelvis with no evidence of free air.The patient underwent emergent exploratory laparotomy and subsequent resection of 55 cm of grossly necrotic small bowel followed by primary enteroenterostomy and end-to-end anastomosis. Copious volume of hemorrhagic fluid was present within the abdomen prior to evisceration of the small bowel.Gross examination of the small bowel showed brown, dusky, and focally granular serosa. The mesentery was markedly firm, fibrotic, and focally retracted the intestinal wall. No clear perforations or fistula were identified. Upon opening, the mucosa was brown and edematous. Serial sections through the specimen revealed hemorrhagic and fibrotic cut surfaces without the presence of a distinct mass. Microscopic examination showed a lobulated to infiltrative vascular neoplasm involving adipose tissue, serosa, muscularis propria, and submucosa . The neoClinically, the patient had normal platelet levels, excluding an association with consumptive coagulopathy leading to thrombocytopenia (Kasabach-Merritt phenomenon). No complications were reported during the procedure or in the immediate "post-operative period", and the patient was discharged after stabilization and recovery of intestinal function with outpatient follow-up planned shortly thereafter. At the time of this report, the patient is stable, with no evidence of disease 5 months after surgery. We opted to manage him expectantly with serial CT scans of the abdomen and pelvis and to monitor for signs and symptoms that would suggest the development of Kasabach-Merritt phenomenon.Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular neoplasm with histological features resembling those of Kaposi sarcoma  and capillary hemangioma mainly presenting in children and adolescents . More thKHE is not associated with HHV8 infection like Kaposi sarcoma; its etiology is unknown . ImmunohKHE is a locally aggressive neoplasm. Exceptionally rare reports of metastasis are present in literature . HoweverAs for the management of patients with KHE, the most critical points to discern are if there is an association with Kasabach-Merritt phenomenon to determine the need for hemostasis and whether the lesion is sizeable and symptomatic enough to warrant treatment. Strategies range from complete surgical excision (which may be difficult given that tumor margins are often poorly defined) to laser therapy and chemotherapy  if tumor is not amenable to resection . The latIn summary, Kaposiform hemangioendothelioma represents an exceedingly rare entity usually affecting children and adolescents with only 4 cases reported in adults in the literature. Our case of a 29-year-old man presenting with intestinal obstruction is unusual with regard to age as well as location and pattern of presentation. An extensive literature search reveals a single instance of KHE presenting as intestinal obstruction involving a sixteen-month-old boy , but no


Lymphatic vascular malformations are rare findings in canine patients with six reports available in veterinary literature. Retroperitoneal cystic lymphatic malformations have not been described previously in canine patients and neither has the use of immunohistochemistry to determine their origin, i.e. vascular versus lymphatic.An 8-year-old neutered female Cocker spaniel was referred for pollakiuria, dysuria and a painful abdomen. Computed tomography scanning of the abdomen showed a fluid filled structure adjacent to the urinary bladder. During surgical exploration, a thin walled cystic structure with sero-haemorrhagic fluid was found, extending from the retroperitoneal space into the abdomen. The mass was excised and submitted for histopathology, revealing a cystic mass lined by a fibrovascular capsule within the retroperitoneal/mesenteric adipose tissue. The inner surface of the cyst was lined by a single layer of bland, flattened spindle cells. Intramural blood vessels were well differentiated, with perivascular haemorrhage. On recurrence 11 months later, the mass was excised for the second time and a PleuralPort  was placed. Fifteen months after initial presentation, progression occurred with haemorrhagic fluid in the cystic space, pleural- and abdominal cavities and the owners opted for euthanasia. Histopathology and positive immunohistochemistry for lymphatic markers lymphatic vessel endothelial hyaluronic acid receptor-1 (LYVE-1) and prospero homeobox protein-1 (PROX-1) confirmed a lymphatic vascular origin of the cystic structure.To our experience, a definitive diagnosis of retroperitoneal cystic malformation of lymphatic origin could be done only by combining the clinical presentation, advanced imaging, histopathology and LYVE-1 and PROX-1 immunohistochemistry. This is the first report of a vascular malformation in a dog where immunohistochemistry was used to make a final diagnosis. A lymphatic malformation, even if rare, should be added on the list of the differential diagnosis in a patient with a retroperitoneal cystic structure containing serohaemorrhagic fluid. Results of this case report can aid in diagnosis of future cases, however, further studies on therapy and management are needed to provide additional information about optimal treatment of these patients. Vascular anomalies can occur due to tumoral cell proliferation or abnormal development of vascular endothelium  . CongeniA review of the veterinary literature suggests that the vast majority of vascular malformations are of blood vessel origin –15. OnlyAn 8-year-old neutered female Cocker spaniel was presented to the referring veterinary surgeon with a 24 h history of pollakiuria, dysuria and a painful abdomen on clinical examination. Lateral abdominal radiograph- and ultrasound examinations showed a large fluid containing structure with intraluminal hyperechoic masses, that was misinterpreted as the urinary bladder. A volume of 300 mL haemorrhagic fluid was drained by cystocentesis and, due to suspicion of a bladder malignancy, the case was referred for further evaluation.Angiostrongylus vasorum) test was negative. Abdominal ultrasound revealed free echogenic fluid which was haemorrhagic on paracentesis with a packed cell volume (PCV) of 17% and total solids (TS) 32 g/L, compared to the peripheral blood which had a PCV of 48% and TS of 66 g/L. This excluded abdominal bleeding and raised suspicion of a cystic mass with occult bleeding within the cyst.On clinical examination the dog was bright and alert with a body condition score of 6/9, normal breathing, pulse 100/min, T 38.8 °C, and pink mucous membranes. The abdomen was painful on palpation. Haematology, biochemistry and coagulation tests  were unremarkable apart from mild hyperkalaemia . To rule out a bleeding disorder, additional tests were performed: the buccal mucosal bleeding time was normal and a lungworm  contrast-enhanced scan was performed. In the thoracic cavity mild atelectasis of no clinical significance was found, while in the abdomen a large volume of echogenic peritoneal fluid, with no contrast enhancement was found Fig. . On the Histological examination of the excised tissue confirmed a cystic lesion composed of a fibrovascular capsule, with an inner layer of bland, flattened spindle cells Fig. . The bloFour months after surgery, an abdominal ultrasound examination revealed a localized multiloculated cystic structure in the same region where the initial cyst had been seen. This cystic structure was approximately half the size of the initial cyst. As the amount of fluid in the cystic lesion was small and the dog was without clinical signs, conservative treatment was opted and a re-check in 3 months’ time was advised.Eleven months after the initial presentation, the dog was presented again with recurrence of a distended abdomen. Abdominal ultrasound revealed progression of the retroperitoneal lesion with a dilated ureter and a moderate hydronephrosis on the left side. Surgical exploration showed recurrence of a large single lumen cystic mass in the left retroperitoneal region, with multiple adhesions to bladder, loops of small intestine and terminal branches of the aorta. Hence, it was not possible to deroof the mass and partially excise the wall as it was the first time. The mass was dissected from the left ureter to relieve the obstruction and the cyst was drained. To be able to provide an entrance port for drainage, an implantable drainage system  was placed. The fenestrated part was placed in the cystic cavity without suturing and the injection port was placed in the left inguinal region. Follow-up consultations were scheduled with 3 months’ interval, to carry out ultrasound guided drainage of the cyst via the PleuralPort.The lesion showed similar histomorphology to the initially excised mass. Immunohistochemistry for lymphatic markers (LYVE-1 and PROX-1) was performed and revealed weak to mild positive intracytoplasmic labelling for LYVE-1 Fig. c and modFifteen months after initial presentation the dog was brought in for routine follow-up. Abdominal ultrasound revealed similar findings to the previous visit, with no further progression. No fluid could be drained from the PleuralPort. As the condition was stable, a follow-up in 3 months’ time was advised. Four days after this routine follow-up, the dog was presented for lethargy and weakness, with a PCV of 14% and TS of 41 g/L. Ultrasound of the abdomen revealed fluid accumulation in the cystic cavity, pleural- and abdominal spaces. Abdominocentesis was performed and haemorrhagic fluid was aspirated with a PCV of 21%. Coagulation tests was within normal limits. The owners did not wish to proceed with further procedures and opted for euthanasia. Post-mortem examination was declined by the owners.Lymphatic malformations in humans tend to occur in infants and are believed to arise from a congenital failure of primitive lymphatic sacs to develop communications with the venous system . MutatioOnly few case-reports about lymphatic malformations have been published in veterinary literature and classification of vascular malformations in veterinary medicine has not been well established due to their infrequency –21. GrosLymphatic malformations in the retroperitoneum and abdominal viscera are a rare entity and account for 2% of all lymphatic malformations in humans . AlthougInitial evaluation of cystic structures by ultrasonography, CT scanning or magnetic resonance imaging is advised, however definitive diagnosis can only be made through histopathology , 31. CT-Invasive or infiltrative growth is commonly associated with malignant behaviour. However, vascular malformations have been reported to have a progressive behaviour and a tendency to expand into surrounding tissues , 25, 26.In humans, the ideal treatment for symptomatic or large retroperitoneal cystic lymphatic malformations is surgical resection to achieve complete excision –39, althDespite documentation of recurrence of the cystic structures in some cases, long term prognosis of lymphatic malformations in dogs is not well-established –21. No hVascular malformations should be taken into consideration in a patient presented with a retroperitoneal cyst containing haemorrhagic fluid. Additional imaging, histopathology and LYVE-1 and PROX-1 immunohistochemistry can be used to diagnose lymphatic malformations.


Ectopic adrenal tissue is the adrenal rests along the path from gonads to adrenal glands during embryogenesis. Ectopic adrenocortical adenoma is a rare disease represented with over-production of cortisol by the ectopic adrenocortical tissue.An 18-year-old Chinese female patient was presented with weight-gain for 6 months. She had elevated plasma cortisol and a solitary mass was revealed using computed tomography scan in the left renal hilum.The tumor was removed and the immunohistochemical profile indicated an ectopic adrenocortical adenoma.After the tumor was removed, the patient was under glucocorticoid replacement therapy in 6-month.During 6-month of follow-up, the patient showed no signs of tumor recurrence.Ectopic adrenocortical adenoma is difficult to diagnose due to its low incidence, and the ectopic rests in renal hilum could be misdiagnosed as renal cell carcinoma. This case reminds clinicians to be aware of ectopic site in the diagnosis of adrenocorticotropic hormone (ACTH) independent Cushing's syndrome. Immunohistochemical stain may assist in evaluating the origin of the ectopic rests. A certain rate of local recurrence indicated the need of long-term follow-up. Ectopic adrenal rests exists along the migration path of adrenal cortex development, and these anatomic sites includes celiac plexus, kidney, broad ligament, testis, and spermatic cord.,3 The hormone secreting status decides the clinical features of ectopic adrenal rests, and those functional rests are symptomatic and more detectable than non-functional ones. Here, we report a case of ectopic adrenocortical adenoma located in the left hilum.The adrenal glands are of a dual embryological origin. The adrenal cortex is derived form the coelomic mesoderm of the urogenital ridge, and the adrenal medulla arises form neural crest tissue.22. Plasma cortisol concentrations were 20.67 μg/dL in the morning , 17.67 μg/dL in the afternoon  and 18.95 μg/dL in the midnight , indicated that the normal circadian rhythm was lost. The morning, afternoon and midnight plasma adrenocorticotropic hormone (ACTH) concentration was 1.32 pg/mL , 1.37pg/mL, and 1.01pg/mL, respectively. Urinary free cortisol (UFC) concentration was 1824 μg/24 h . During the low dose dexamethasone depression test (LDDST), the morning plasma cortisol slightly increased from 20.67 μg/dL to 25.66 μg/dL, and the UFC after oral dexamethasone was 1388.8 μg/24 h. The patient underwent a high dose dexamethasone suppression test (HDDST), the morning plasma cortisol was 27.52 μg/dL and UFC was 1726.1 μg/24 h after oral dexamethasone intervention. Both morning plasma cortisol and UFC in LDDST and HDDST were not suppressed, supporting the diagnosis of ACTH-independent Cushing's syndrome. Adrenal computed tomography (CT) scan revealed a well-circumscribed round mass with a maximum diameter of 3.0 cm in the left renal hilum, and bilateral adrenal glands were atrophic  and blood glucose remained normal . Her body mass index was 26.6 kg/mThe patient underwent laparoscopic resection of the tumor. During the operation, the mass in the left renal hilum was completely resected. Postoperative pathology results confirmed the diagnosis of ectopic adrenocortical adenoma. The patient was followed up for 12 months after the operation. She was under glucocorticoid replacement therapy for 8 months, and hydrocortisone 100 mg was intravenously given in the operating day and 2 days after the operation. Oral prednisone (10 mg bid) was given 7 days after the operation for a week and gradually tapered for 8 months duration according to clinical symptoms and morning plasma cortisol. No tumor recurrence or metastasis was found after 12 months of follow-up.2.1The tumor was in size of 3.0cm × 2.5cm × 1.5 cm. Grossly, the mass was round, solid and well circumscribed. Its cut surface was brown  supported an adrenocortical origin rather than a renal or mesenchymal origin, which was in accordance with preview reports. The confirmation of malignancy depends on tumor behavior of malignancy, such as distant metastasis and invasion, which suggests the critical role of post-operative follow-up.The differential diagnosis from adrenocortical adenocarcinoma is of great importance. In our case, the imaging of the tumor was not as large in diameter as most adrenocortical adenocarcinomas and showed no features of malignancy, including capsular or vascular invasion, necrosis, and distant metastasis. Histological stain of the tumor could also evaluate the malignancy of adrenocortical tumors. Histological signs of malignancy include necrosis, abnormal mitosis and capsular invasion, as was summarized in Weiss criteria.,12 Other ectopic adrenal rests were not found clinical evidence of recurrence, while the duration of follow-up was no long enough for some of the cases. In Cushing's disease and adrenocortical adenoma, there is possibility of recurrence and long-term follow-up is recommended. For ectopic adrenocortical adenoma, long-term follow-up consists of surveillance of morning plasma cortisol and imaging examination, such as CT, to rule out local recurrence.The treatment of functioning ectopic adrenal tumors is radical resection. Otherwise, watchful waiting is a choice for nonfunctioning ones. After surgery, long-term follow-up is of great importance due to the possibility of recurrence. In the previous reported cases, 2 patients appeared symptoms of Cushing's syndrome, and local recurrence of adrenocortical adenoma was diagnosed.4This case provides an extremely rare case of ectopic adrenocortical adenoma in the left renal hilum to clinicians as a reminder of the scarce situation. Pre-operative biopsy and immunohistochemical staining could help in diagnosis and confirming the origin of the tumor. Long-term follow-up is necessary in both plasma cortisol and imaging examination due to the possibility of local recurrence.We thank the patient who agreed to our report with anonymity and for providing a detailed medical history.Data curation: Difei Lu.Formal analysis: Difei Lu.Investigation: Difei Lu.Methodology: Nan Yu.Supervision: Nan Yu.Validation: Difei Lu.Visualization: Difei Lu.Writing – original draft: Difei Lu.Writing – review & editing: Nan Yu, Xiaowei Ma, Xiaohui Guo, Junqing Zhang.Difei Lu orcid: 0000-0003-3953-8900.


Primary hepatic neuroendocrine tumours (PHNET) were first described by Edmondson et al. in 1958 and are rare, accounting for only 0.3% of all neuroendocrine tumours. Only several hundred cases have been reported.We present two cases. The first is a 65-year-old asymptomatic male referred with a liver lesion on ultrasound performed to investigate a mildly elevated Alanine Aminotransferase (ALT). Hepatitis serology and tumour markers were normal. He had an unremarkable colonoscopy and gastroscopy. CT and MRI revealed a single liver lesion adjacent to the gallbladder suspicious for malignancy. He underwent a segment IVb/V liver resection. Histology was consistent with a 65 mm grade 2 PHNET. Subsequent Dotatate PET/CT scans have been normal at 5 years. The second is an asymptomatic 73-year-old male referred with fluctuating hepatic enzymes and a history of alcohol overuse. Imaging revealed a suspicious lesion in segment III of the liver. He underwent a left lateral liver resection. Histology revealed an 18 mm grade 1 PHNET. A subsequent Dotatate PET/CT was normal with no new disease at six months.PHNET, albeit rare are in the differential diagnosis for primary hepatic malignancies. Tumour markers are usually normal and radiological imaging can mimic other hypervascular hepatic tumours. Surgery is the only curative treatment for localised disease to date.PHNET needs to be considered in asymptomatic patients with hypervascular hepatic lesions. More research is required before other adjunct treatment options can be suggested. It repr2Registration and Ethics: the case series is registered on Research Registry (UIN: researchregistry4623). Ethics approval from the hospital’s Human Research and Ethics Committee (HREC) was obtained (protocol 18/43). All procedures performed were in accordance with the ethical standards of the institutional and the 1964 Declaration of Helsinki and its later amendments. Written informed consent was obtained from the patients for publication of this case series and accompanying images. A copy of the consent is available for review by the Editor-in-Chief of this journal on request.2.1The case series is a single-centered, retrospective and consecutive in design.2.2The patients were managed in a private practice setting.33.1A 65-year-old man was referred in April 2013 with an echogenic segment IVa lesion found incidentally on ultrasound scan (USS). He was being investigated for fatty liver disease due to an elevated ALT (45 U/L). The patient consumed one bottle of wine daily with a background of hypertension, dyslipidaemia and gastroesophageal reflux disease. The patient was asymptomatic with a normal abdominal examination. His bloods and tumour markers including carcinoembryonic antigen (CEA), cancer antigen (CA) 19-9 and alpha-fetoprotein (AFP) were normal. A colonoscopy and gastroscopy was performed revealing only non-specific duodenitis with no malignancy.A triple phase computed tomography (CT) scan identified a bilobed exophytic 70 × 56 x 78 mm segment IV liver lesion adjacent to the gallbladder and displacing the cystic artery. There was heterogenous hyperenhancement during arterial phase with washout in portal venous (PV) phase and no delayed enhancement. A magnetic resonance cholangiopancreatography (MRCP) showed the lesion had a multi-cystic or necrotic component posteriorly measuring 18 mm and was hyperintense in both T1 and T2-weighted imaging (WI) without fat or calcification. There was arterial enhancement that persisted into the PV phase without delayed phase enhancement. The right portal vein and its segmental branches were draped around the lesion causing mild compression, while the segmental biliary tree distal to the mass was dilated. The patient did not undergo a pre-operative biopsy. The differential diagnosis was hepatocellular carcinoma (HCC) in a non-cirrhotic liver, lymphoma or primary gallbladder tumour.The patient underwent a segment IVb/V liver resection with enbloc cholecystectomy by a specialist hepato-pancreato-biliary (HPB) surgeon. The small bowel was normal. The mass is illustrated in The patient underwent a Dotatate positron emission tomography (PET)-CT scan post-operatively which revealed mild uptake in the head and uncinate process of the pancreas. This was considered to be a normal physiological variant given a normal CT scan pre-operatively. The Dotatate PET-CT scan was repeated six months later revealing a similar but stable appearance in the pancreas. Annual follow-up with CT scans for the last five years have been reassuring with no signs of recurrence or metastatic disease.3.2A 73-year-old male was referred with mildly deranged liver enzymes. The patient was a heavy drinker, consuming 100 g of alcohol per day for four decades. He had a background of heterozygous haemochromatosis, pacemaker and a transurethral resection of the prostate. The patient was asymptomatic with a normal examination. His hepatocellular liver enzymes were minimally elevated initially but had normalised on repeat testing a month later. Tumour markers including AFP, CA 19-9 and CEA were all normal. The patient had a normal colonoscopy two years prior.An USS revealed hepatomegaly with a solid 25 mm hypoechoic area in segment III with a cystic component. The CT scan showed a 20 mm segment III liver lesion with enhancement in arterial and PV phase and washout on the delayed phase with an enhancing capsule. Due to the alcohol history the possibility of HCC in a cirrhotic liver was suspected. After a satisfactory indocyanine green clearance test, the patient underwent an uncomplicated left lateral liver resection by the same specialist HPB consultant surgeon. The patient was not macroscopically cirrhotic and there were no lesions in the small bowel. The tumour measured 18 × 17 mm. Microscopically, there was mild hepatic steatosis with no fibrosis. IHC staining for synaptophysin and chromogranin were positive. There were no mitoses but a Ki67% proliferation index of 0.5% was consistent with a well-differentiated grade 1 NET. IHC stains for HepPar 1, CD10, TTF-1, CDX2 and PAX8 were negative.The patient underwent a Dotatate PET-CT scan two months post operatively to search for an undiagnosed primary and this was normal. Repeat Dotatate PET-CT 6 months later was again normal. Given the patient had a colonoscopy only two years prior and there were no other CT or PET-CT signs of extra-hepatic disease, a final diagnosis of HPNET was made.4PHNETs are more frequent in females 58.5%)  with a m.5%  withThe diagnosis of PHNET requires a histopathological specimen as well as exclusion of an extrahepatic primary lesion  because USS of PHNETs can appear hypoechoic, hyperechoic or mixed in appearance. Li et al. reported that 60% of their case series displayed intralesional blood vessels compared to only 26–28% in metastatic liver NETs . CT scanMacroscopically, PHNETs appear as solid lesions that can have cystic components  with a gSurgery is currently the only curative treatment for localised PHNET with 5-year survival rates ranging from 74 to 78% and a recurrence rate of 18% . Liver t5Little is known about the clinical course of PHNETs because of the scarcity of cases. Bloods and tumour markers are non-specific and radiological findings mimic other hypervascular hepatic lesions. More studies are needed to investigate the effectiveness of TACE and chemotherapy on PHNETs but this may be difficult given the rarity. PHNETs should be considered in the diagnosis and management of all hypervascular hepatic masses, especially in patients with no chronic liver disease and normal tumour markers.Nil.Nil.Ethics approval from the hospital’s Human Research and Ethics Committee (HREC) was obtained (protocol 18/43). All procedures performed were in accordance with the ethical standards of the institutional and the 1964 Declaration of Helsinki and its later amendments.Written informed consent was obtained from the patients for publication of this case series and accompanying images. A copy of the consent is available for review by the Editor-in-Chief of this journal on request.Nelson Chen: original draft, review and editing, final approval of version to be submitted.Kellee Slater: conceptualisation, review and editing, revising critically for important intellectual content and final approval of version to be submitted.Name of research: Research Registry.Date of registery: 11 January 2019.URN: researchregistry4623.Nelson Chen and Kellee Slater.Not commissioned, externally peer-reviewed.


This case report documents the diagnosis, treatment, and outcome of a nonresectable oral squamous cell carcinoma in a dog with initial poor prognosis. An approximately 4-year-old female Staffordshire Bull Terrier presented with a large mass on the front of lower jaw which was diagnosed as oral papillary squamous cell carcinoma by histopathology. CT scans revealed invasion of the cancer to the frenulum of the tongue. The mass was inoperable due to location, expansiveness, and metastatic lymph nodes. The dog received 4 treatments of intralesional hyaluronan-platinum conjugates  at 3-week intervals. Clinical chemistry and complete blood count were performed one week after each treatment and results were within normal limits. Complications included bleeding due to tumor tissue sloughing, as well as a single seizure due to unknown causes. Upon completion of chemotherapy, CT showed that the mass had regressed and was no longer invading the lingual frenulum, and multiple lymph nodes were free of metastasis. The mass thus became resectable and the dog successfully underwent rostral bilateral mandibulectomy. Over one year after chemotherapy and surgery, the cancer remains in complete remission. HylaPlat  is an investigational platinum-based chemotherapeutic using hyaluronic acid as a carrier and delivery agent. In our previous studies, HylaPlat has been safely administered intralesionally to dogs and other preclinical animals without any dose-limiting nephrotoxicity, the primary side effect of intravenous cisplatin chemotherapy. Intralesionally administered HylaPlat does not result in extravasation as seen in intralesional cisplatin . HylaPlaAn approximately four-year-old female intact Staffordshire Bull Terrier was presented for evaluation of a large and fast-growing mass on the front of lower jaw, involving several teeth . The dogA large ulcerated mass was present on the rostral portion of the mandible. Full mouth dental radiographs revealed a mass that encompassed all of her lower incisors as well as her left canine and premolars. Her abdomen palpated soft and nonpainful. On palpation, her submandibular and prescapular lymph nodes were enlarged. The dog was anesthetized and a computed tomography (CT) scan of the head was performed with contrast. The CT scan revealed a large, interosseous, and expansile soft tissue attenuating mass at the most rostral aspect of the mandible, involving teeth, jaw bone, and oral membranes, and expanding almost to the frenulum of the tongue. There was a bilateral symmetrical enlargement of the mandibular lymph nodes with moderate heterogeneity following contrast. Both retropharyngeal, both prescapular and right superficial cervical lymph nodes were also enlarged. Histopathology of tissues from the mandibular mass was consistent with papillary squamous cell carcinoma. Cytology of the submandibular lymph nodes indicated reactive lymphoid hyperplasia and neoplasia. Because the mass was quite expansive and the cancer had metastasized, surgery intervention with a goal of achieving 12-15 mm margins without involving the frenulum was impossible2 (mg of chemo per m2 of body surface area). The dog weighed 50.8 ± 1.2 lbs throughout the entire study. All four treatments went smoothly and recovery after sedation was uneventful. One hour after the first treatment, a blood sample was collected to determine the systemic exposure of the medication. Clinical chemistry and complete blood counts were performed approximately biweekly between treatments to evaluate the tolerability of HylaPlat and the results were compared to the prestudy values. Specifically, the blood samples were collected on days 0 (prior to the study), 8, 18, 26, 30, 36, 40, 47, 51, 55, 58, 61, and 68. Prior to each treatment, tumor size was measured using a caliper to monitor patient's response to the chemotherapy.Shortly after diagnosis, the dog was accepted into the HylaPlat chemotherapy study sponsored by HylaPharm . The dog received four intralesional injections of HylaPlat under anesthesia at three-week intervals on days 1, 22, 43, and 64. The doses ranged from 5 to 10 mg/mClinical chemistry indicated that liver enzymes  were within normal limits during the entire study except on day 58 when AST and ALT were temporarily elevated and over the upper limit . All three liver enzymes returned to normal range by day 68 . Total bBecause platinum chemotherapy may induce tubular injuries and may cause nephrotoxicity, renal function was monitored by BUN and creatinine tests during the study to assess systemic tolerability . Test reA complete blood count with differentials was performed to determine the patient's general health status. Specifically, neutrophil and hematocrit counts are monitored to determine whether chemotherapy significantly affects hematopoietic effects and myelosuppression. Platelet count is also evaluated as myelosuppression is one of the systemic intolerabilities of platinum-based chemotherapies. Results indicated that neutrophil counts at pre- and posttreatments were not statistically different , suggesting that HylaPlat chemotherapy did not result in bone marrow suppression for this patient . Similar2. The mass measured 5.2 cm by 5.1 cm by 3.3 cm on the day of the first treatment. After the first treatment, owner reported that the dog ate well and acted normally and no side effects were observed. Prior to the treatment the front of the tumor bled easily and after the first treatment the bleeding stopped. Three weeks after the first treatment, the dog returned for the second injection. The attending veterinarian reported that the dog's left submandibular lymph node was not as enlarged as before the first treatment on palpation. The tumor measured wider across (7.1 cm by 5.4 cm by 3.9 cm); however, the appearance of the mass improved visually with less purulent discharge and less observed bleeding. After the second treatment, the dog ate well and acted normally. Necrotic pieces of tumor tissues were observed to slough off days after the treatment. The exposed blood vessels caused by the sloughing tissues resulted in bleeding. The bleeding was managed by administration of a tranquilizer (acepromazine) to lower blood pressure and reduce activity. The dog responded to the medication and the bleeding stopped. On the day of the third treatment, the tumor measured slightly smaller on two dimensions and slightly larger on the third dimension (6.7 cm by 5.6 cm by 3.8 cm) compared to measurements made at the second treatment. After the third treatment, the owner reported that more tumor tissues fell off and the dog had heavy bleeding from the sloughing mass for 3 days. The bleeding complication may be associated with the effect of the chemotherapy  as well as the friable nature of the tumor . Besides these symptoms, the dog acted healthy and ate well. Three weeks later, the dog received the fourth and the last injection. The tumor measured slightly smaller on two dimensions and slightly larger on the third dimension (6.5 cm by 5.5 cm by 4.8 cm) compared to measurements made at the third treatment, which we considered to be stable. Clinically, the patient was doing well; however, the tumor did bleed when it was debrided. The owner reported that the dog ate well and did well after the fourth treatment. On day 7 after the fourth treatment, heavy bleeding began from the posterior of the tumor. The dog had a single, brief seizure, the cause of which was unknown, though considerations may include blood loss, idiopathic epilepsy, liver disease, kidney failure, brain tumor, and other possible seizure-triggering conditions. The dog responded to diazepam and seizure did not recur (Grade II AE per VCOG-CTCAE). Additional diagnoses were not performed. After recovery from the ictal period, the dog was assessed as neurologically healthy.The patient received four treatments of HylaPlat at 3-week intervals. The doses were 5, 7.5, 10, and 7.5 mg/mApproximately one month after the last treatment, radiographs, CT, lymph node biopsy, and a physical exam were performed to determine the status of the tumor and evaluate the feasibility of a mandibulectomy to remove the SCC. Chest radiographs suggested no evidence of metastasis to the lungs. CT of the head demonstrated that the margins of the mass had regressed compared to the CT prior to the chemotherapy and were no longer invading the lingual frenulum . HistopaThe dog was placed under general anesthesia and underwent surgery to have an enlarged lymph node and the SCC removed. Surgery went as planned and without complication. She was laid on her left side and an incision was made on the right side of her neck to have the enlarged retropharyngeal lymph node removed. Then she was placed on her back and had most of her lower jaw removed in an effort to achieve clean margins around the SCC. The jaw was cut just in front of her first molar on the left side and just behind her third premolar on the right side. She recovered uneventfully following surgery.Histopathology of the mandibular mass indicated that soft tissue excision was complete with clean margins. Histopathology of the right retropharyngeal lymph node was consistent with the reactive lymph node. In the days following surgery, the dog had moderate-to-severe edema and swelling around her surgical sites, which may be resulted from disruption of the lymphatic system considering the surgical locations, inflammation caused by the rostral bilateral mandibulectomy, or possible infections of the tissues and the mandibular bone at the surgical site. In an attempt to prevent infection at this site, a short course of antibiotics  was recommended. The dog was present 4 weeks after the surgery and a physical exam indicated that the healing of the surgical site was satisfactory . Over onHylaPlat  is an injectable chemotherapy for treatment of locally advanced solid tumors, including oral SCC which is one of the most common head and neck cancers in dogs. In preclinical studies, HylaPlat has demonstrated anticancer activity against a wide range of neoplasms in mouse xenografts, including head and neck cancer , 7, breaAccording to the attending veterinarians, the lyophilized HylaPlat was readily rehydrated using sterile water in a few seconds and the intralesional injections using 23 or 25 gauge needles went smoothly without complication. To minimize environmental contamination, the medication was placed in a sealed and stoppered glass vial. Prior to use, the medication was rehydrated using Water-For-Injection by piercing the stopper. Once completely reconstituted, a proper dose was withdrawn from the vial using a syringe with a Luer Lock needle connection. The attending veterinarians and technicians were required to wear personal protective equipment (PPE) such as gloves when handling the medication.2 during the third treatment. Platinum-based chemotherapy regimens such as cisplatin and carboplatin are known to induce thrombocytopenia when used alone or in combination with other chemotherapeutic agent. Possible causes of chemoinduced thrombocytopenia may be due to the effects of the medication on stem cell proliferation, megakaryocyte progenitor cell apoptosis, platelet production, and other underlying changes in the bone marrow [During the course of the treatment, the patient did not experience any intolerability relating to nephrotoxicity, which is the dose-limiting toxicity of platinum-based chemotherapy. The result was consistent with previous findings from rodent, rabbit, and canine preclinical studies and canine clinical studies –3, 6, 8.e marrow –13. The Oral squamous cell carcinoma is the second common oral cancer in dogs. In general, tonsillar SCCs are more aggressive and metastatic than nontonsillar SCCs. Prognosis depends on the location of the tumor and the status of regional and systemic metastases. Treatment regimens depend on the location and the stage of the cancer, which include surgery with 12-15 mm margins, radiation therapy, and chemotherapy. Surgery remains the first option if the tumor is resectable and cancer has not spread to regional lymph nodes. Radiation therapy and chemotherapy are the preferred choices if cancer has metastasized or tumor is nonresectable due to either the location or the degree of invasiveness.In this case, the patient was nonoperable as the expansive rostral mass on the mandible extended to the frenulum of the tongue, making complete surgery excision with clean margins impossible without the involvement of the tongue. Furthermore, the cancer has metastasized to the regional lymph node at the time of the diagnosis, resulting in possible poor prognosis even if partial rostral bilateral mandibulectomy was successfully performed. Thus, neoadjuvant chemotherapy was recommended with the objective of reducing both the primary mass and lymphatic penetration prior to surgical SCC removal. After four HylaPlat treatments, the primary lesion decreased in size and moved away from the base of the tongue according to the CT result, enabling surgical excision of the tumor. In addition, HylaPlat delivered the cytotoxic platinum in a sustained manner to the metastatic lymph nodes via lymphatic drainage of the primary tumor due to its engineered size (25 nm) and CD44-associated high affinity to cancer cells in the lymphatic micrometastases , 15. In


The involvement of granulomatosis with polyangiitis is less frequent in the intestine.We present a case of Wegener’s granulomatosis with unusual endoscopic appearance, involvement in a young man’s gastrointestinal tract. A 45-year-old man was diagnosed with Wegener’s granulomatosis 11 years ago, and relapsed with abdominal pain and melena. A colonoscopy was performed, and the appearance of mucosal lesions with an unusual annular black membrane was observed. A black ring-shaped membranous tissue adhered to the surface of the colon wall, which could be traversed by an endoscopic forepart.Biopsy of the black membrane revealed degenerative colonic mucosal tissues, while deep colonic biopsy revealed inflammatory granulation tissues. This has not been reported in previous documents. Granulomatosis with polyangiitis (GPA), which is also known as Wegener’s granulomatosis (WG), is a necrotizing granulomatous vasculitis with small arteriovenous and capillary involvement . At presA 45-year-old male was hospitalized due to bloody nasal discharge, hemoptysis and rash for 11 years, which aggravated after 2 weeks, and presented with abdominal pain and melena for 1 month. The patient was hospitalized and diagnosed with WG 11 years ago. Furthermore, he had rashes on his face, trunk, limbs and feet, and had oral ulcers, perianal ulcers, and sinusitis.9/L, hemoglobin level of 79 g/L, urine protein (+), microhematuria (+), erythrocyte sedimentation rate of 50 mm/h, c-ANCA (antineutrophil cytoplasmic autoantibodies) with a titre of 1 in 100, and PR3-ANCA (anti-neutrophil cytoplasmic antibodies proteinase 3) of > 200 RU/ml. Computed tomography (CT) revealed left frontal and ethmoid sinusitis, and bilateral maxillary sinusitis. Based on these clinical features and laboratory findings, the patient’s diagnosis of WG was accurate. In order to clarify the cause of the abdominal pain, a colonoscopy was performed with the written informed consent of the patient. Different sizes of irregular ulcerations, which were 3–4 cm and 1–2 cm in diameter, were scattered at different intervals throughout the colon (Fig. The laboratory tests revealed the following: leucocytes count of 8.5 × 10lon Fig.  and b. Tlon Fig.  and d. OGranulomatous vasculitis is a necrotizing small-vessel vasculitis, and granulomatous inflammation is strongly associated with anti-neutrophil cytoplasmic autoantibodies . The typPagnoux et al.  conducteAlthough the present understanding of gastrointestinal lesions in GPA patients has increased, there are few reports on the endoscopic appearance of GPA involving the bowel with accompanying images. In previous years, Robin et al.  reportedIn conclusion, when GPA patients suffer from abdominal pain, diarrhea, bloody stools, or melena, the differential diagnosis should be made while taking into account the possibility of granulomatous vasculitis involving the gastrointestinal tract. Colonoscopy combined with tissue biopsy can help to determine the nature of the disease, and provide an effective method for differential diagnosis.


Primary lymphoma of the oral cavity is a rare phenomenon. Herein we describe a unique presentation of lymphoma of the tongue which initially manifested as trigeminal neuralgia.A 63-year-old female experienced a 10-month history of paresthesia and neuropathic pain involving the left tongue and mandibular area of her face. Investigations including imaging and biopsy revealed primary lymphoma of the tongue with extensive perineural spread. The patient underwent standard chemotherapy with complete radiological response, but minimal recovery of the affected neurological functions.This case highlights an unusual presentation of a rare disease leading to a delay in diagnosis and the importance of a complete workup for trigeminal neuropathy. The most common malignancy in the oral cavity is squamous cell carcinoma, which typically develops as a red or white lesion of the oral mucosa. Lymphomas are the most common non-epithelial malignancy in the oral cavity, though considered a rare entity, representing 3–5% of all reported cases of lymphoma . The mosA 63-year-old previously healthy Caucasian woman was evaluated for a 10-month history of paresthesia and neuropathic pain involving the left tongue and left mandibular area of her face. She was initially treated for presumed trigeminal neuralgia, and neuropathic pain agents helped her marginally. Due to a lack of response to treatment, a magnetic resonance (MR) scan with gadolinium contrast of her head was ordered by an otolaryngologist. The scan showed abnormal enhancement in the left Meckel cave along the course of the mandibular nerve with involvement through the foramen ovale, inferior temporal fossa, and medial pterygoid muscle. She was referred to a neuro-oncologist due to concerns that her neuropathy was related to metastases. Computed tomography (CT) scans with intravenous and oral contrast of the head/neck, thorax, and abdomen/pelvis initially did not show evidence of malignancy. During the course of the investigations, the patient was found to have a left-sided tongue mass on physical examination. She was referred to an otolaryngology-head and neck surgeon for work-up of her tongue lesion.The patient did not recall the mass being present prior to her seeing the neuro-oncologist. She denied any pain associated with the mass. Review of systems, including constitutional symptoms, was otherwise negative. Examination of the head and neck demonstrated numbness of her left tongue and left mandibular area of her face. The patient did not report any changes to her sense of taste or hearing. Tongue and facial movement were preserved bilaterally. There was no facial droop. Intraoral examination did not reveal any visible masses or mucosal changes. Palpation of the tongue demonstrated a 1 × 2 cm mass deep to the mucosa that felt rubbery without overlying mucosal changes. The tonsils and uvula were normal. Lymphadenopathy of the head and neck were not appreciated on exam.An incisional biopsy of the tongue mass was performed in clinic, which was read as diffuse large B cell-lymphoma . A gadolinium contrast-enhanced MR scan of the neck was ordered to evaluate the lesion, which showed that the tongue mass exhibited perineural spread along the left lingual and inferior alveolar nerve, tracking along the V3 trigeminal branch to the left Meckel cave, in addition to perineural spread of the left facial nerve along the anastomosis with the auriculotemporal branch with the trigeminal nerve Fig. . It was Incidentally, a mammogram  and biopsy showed that she also had a synchronous invasive mammary carcinoma of the right breast.She was referred to hematology and general surgery for management of both primaries. After a multidisciplinary discussion, it was decided that she would undergo R-CHOP  chemotherapy for her lymphoma prior to definitive management of her breast cancer. She has currently completed 4 cycles of chemotherapy and positron emission tomography demonstrates complete disease response Fig. . FollowiLymphomas are the fifth most common cancer in Canada , and areThe trigeminal nerve provides extensive sensory innervation to the face. The most well-known trigeminal nerve disorder is trigeminal neuralgia, which is classically defined as paroxysmal neuropathic pain along one or more of the trigeminal nerve distributions. The etiology in 80–90% of patients is a vascular anomaly which causes compression of the trigeminal nerve vasculature as it exits the pons. However, other conditions can produce similar symptoms, including malignancy, congenital malformations and multiple sclerosis. These non-classic conditions tend to cause neurologic symptoms in addition to the typical paroxysmal pain pattern . In a joPerineural invasion (PNI) is an important mechanism of malignant spread, in addition to direct invasion, hematologic and lymphatic spread. The mechanism of PNI is complex, involving a reciprocal relationship between host and tumour, in which a multitude of molecules in the nerve microenvironment facilitate metastasis . PNI is To our knowledge, this is the first case report of an oral cavity lymphoma presenting as trigeminal neuropathy. This case highlights the importance of a complete workup for trigeminal nerve disorder, as there is a broad differential for this condition. It also demonstrates an uncommon presentation for lymphoma, the fifth most common cancer in Canada.


To report a rate case of Juvenile xanthogranuloma in a newborn infant. We present the case of a 31-week preterm newborn with multiple skin lesions whose clinical, histological and immunohistochemical findings allowed the diagnosis of juvenile xanthogranuloma. Currently, the patient has nine months-old, and there is no aggravation of the skin lesions or evidence of extra-cutaneous involvement, particularly ophthalmic. Juvenile xanthogranuloma is a rare and benign condition, included in the vast group of non-Langerhans histiocytosis. It typically occurs in the pediatric age and may have a neonatal presentation. It affects predominantly the skin, in the form of papules or yellow and/or erythematous nodules and could be asymptomatic, multiple or solitary. Extra-cutaneous involvement, is more common in toddlers and when multiple lesions are present. The eye is the most affected site. We highlight this clinical case by its presentation in the neonatal period and in the form of multiple lesions, which bestows an increased risk of extra-cutaneous involvement, although this has not yet been verified. The purpose of this case report is to describe a rare case of juvenile xanthogranuloma in the neonatal period.Newborn male with gestational age of 31 weeks, born from a gestation that had prenatal follow-up and no intercurrences. Son of healthy, non-consanguineous parents. At birth, anthropometry and the objective examination were compatible with gestational age.th day of life, there were two nodular, firm, yellowish lesions with no inflammatory signs or ulceration, with 10 mm in diameter (On the subject’s third day of life, an erythematous lesion with about 10 mm in diameter appeared on the left flank . Subsequdiameter .A cutaneous biopsy of the lesion located on the left flank was performed , whose hFaced with this result, the study was completed with transfontanelar, abdominal and bladder-kidney echography, which revealed no alterations. Ophthalmologic observation excluded the presence of ocular lesions.Currently, the patient is 9 months old, the cutaneous lesions are stable and the evolution of weight and length is adequate. Regular neonatology, dermatology, pediatric hemato-oncology and ophthalmology follow-up in consultations was maintained.,,,,,,,,JXG is the most frequent form of NLH..,,,,,,Lesions appear in the first two years of life: 5 to 17% are present at birth and 40 to 70% appear during the first year.,,Because of its benignity and transitory character, it is estimated that it is an underdiagnosed entity and, therefore, of unknown incidence.,,,,,,,,,,,,The most frequent presentation is in the form of a solitary, exclusively cutaneous lesion. It appears as a well delimited, firm, elastic, rounded papule or nodule with 0.5 to 2.0 cm in diameter.,,Extracutaneous involvement is described, and is estimated to occur in 4% of cases, affecting any organ or tissue.,,,,,,,Ocular lesions are the most frequent, occurring in 0.3 to 0.5% of cases. It is mostly unilateral, with the iris being the most affected site, followed by the eyelid. Orbital involvement is rare and predominates in the neonatal period.,,,,,,,,,,,,The involvement of other organs such as the lung, liver, spleen, pericardium, bone, central nervous system, among others, with different systemic repercussions, is also described.,,,,,,,,,,Diagnosis is fundamentally clinical. The differential diagnosis may include histiocytoses of Langerhans cells, Spitz nevi, urticaria pigmentosa, among others. In dubious cases, cutaneous biopsy should provide clarification. ,,,At the time of diagnosis, a meticulous objective examination should be performed to determine the existence of systemic involvement. As extracutaneous involvement is rare, there is no consensus as to whether it should be excluded by analytical and/or imaging evaluation in the absence of suggestive signs or symptoms. Nevertheless, regular ophthalmologic assessment is recommended in children younger than two years of age and with multiple skin lesions.,,,,,,,,,The prognosis of patients with exclusively cutaneous involvement is excellent, with spontaneous remission in months or a few years, and relapses are rare.It can be concluded that JXG is a rare and benign disease, which typically occurs in pediatric age and is underdiagnosed. This clinical case was highlighted by the presentation in the neonatal period and in the form of multiple lesions, which gives it an increased risk of extracutaneous involvement, although this has not been verified. We emphasize the frequent need for histological confirmation and the importance of the ophthalmologic follow-up of these patients.


Pyloric gland adenomas (PGAs) are rare precancerous tumors typically arising from the stomach. Even more rarely do they arise in extragastric sites such as the duodenum and gallbladder. The identification of PGAs is important because they possess a risk of developing into invasive adenocarcinoma. This case report describes a 59-year-old male who presented to our office for a follow-up of a nonspecific duodenal lesion. Endoscopic ultrasound and pathology revealed a PGA with low grade dysplasia and some areas of focal high grade dysplasia. It is important that gastroenterologists are aware of these lesions and their risk of malignant transformation. More studies are needed to describe the long-term behavior of pyloric gland adenomas and to recommend appropriate endoscopic surveillance of these lesions. Pyloric gland adenomas (PGAs) are rare precancerous tumors typically arising from the stomach. They account for about 2.7% of all gastric polyps. Even more rarely do they arise in extragastric sites such as the esophagus, gallbladder, bile duct, duodenal bulb, and duodenum. These lesions are most common in females in their 8th decade of life , 2 likel en bloc using a hot snare following a 4-mL saline lift. Two endoclips were placed for hemostasis. Pathology revealed a pyloric gland adenoma (Figures A 59-year-old Asian male presented to the gastroenterology office for a follow-up of a previously found duodenal lesion. He had an EGD at an outside facility 2 years earlier for evaluation of abdominal pain which showed a 1.2 cm duodenal polypoid lesion in the second portion of the duodenum. Pathology of the polyp revealed heterotopic gastric mucosa with benign lobules of gastric glands. He also was found to have chronically active H. pylori gastritis with numerous Helicobacter organisms and atrophic gastritis with patchy areas of intestinal metaplasia. At that time, he was instructed to follow up for a repeat endoscopy for surveillance in two years. As recommended, two years later he presented to our office for surveillance of his duodenal lesion. Due to his history of atrophic gastritis and a large duodenal lesion, we performed an upper endoscopy that showed atrophic gastritis, duodenitis, and a single 10 mm polyp in the second portion of the duodenum. The duodenal polyp was biopsied. Pathology revealed polypoid gastric metaplastic mucosa with focal epithelial atypia including nuclear enlargement, stratification, nucleoli, and few mitoses. The patient was referred for endoscopic ultrasound to further characterize the lesion and complete resection. Several weeks later he underwent an endoscopic ultrasound that revealed a 13-mm pedunculated and sessile polyp on the lateral wall opposite to the major papilla in the second portion of the duodenum . The pol Figures  and somePyloric gland adenomas are rare neoplasms of the gastrointestinal tract. They are typically found in the stomach but can also originate from the duodenum, gallbladder, bile duct, pancreas, rectum, and heterotopic gastric mucosa in Barrett's esophagus. They have also been reported in the cervix. According to the Vieth study in 2003 in a registry of 2778 gastric polyps diagnosed at the Institute of Pathology of Bayreuth between 1990 and 2000, only 2.7% were found to be pyloric gland adenomas. In the same study, 61% of the adenomas were found in females and the average age for females was 75 [ H. pylori associated gastritis in 30.2%, and only 3.8% of those with normal gastric mucosa [Pyloric gland adenomas have also been reported in association with autoimmune gastritis in 33.9% of patients, activec mucosa . SimilarAccording to the Chen study performed in the US between 2004 and 2008, 36% of patients diagnosed with PGA had no dysplasia, 12% had low grade dysplasia, 39% had high grade dysplasia, and 12% had invasive carcinoma. In the Vieth study performed in Germany between 1990 and 2000, 30% of pyloric gland adenomas were found to have invasive carcinoma. Although there is a discrepancy in the rate of transformation into carcinoma, it has been well-established that there is a significant risk of becoming cancerous. There may be a discrepancy due to the expertise in pathologists diagnosing these lesions. PGAs were first described in Europe and have recently become more recognized in the US. There are currently no specific guidelines for the removal and surveillance of pyloric gland adenomas. However the ASGE GI Endoscopy Guidelines from April 2006 recommend that adenomatous gastric polyps be resected and surveillance endoscopy be performed at 3-5-year intervals [