Primary hydatid cyst of the spinal canal is extremely rare. We reported a 42-year-old Kazakh man with right lower back pain and weakness in both lower limbs for 2 months, who lived in the pastoral area. Clinical examination revealed that the patient had no cysts on other organs and no previous medical history except for a huge cyst inside and next to the vertebrae. MRI examination revealed a huge dumbbell-shaped primary cyst across the intervertebral foramen. Pathological examination after operation confirmed a fine-grained hydatid cyst disease. Therefore, in the pastoral area, doctors should be alert to the occurrence of hydatid cyst disease if patients complained about progressive back pain and lower limb weakness or other spinal cord compression symptoms. Once hydatid cysts in other organs or systems were detected, the occurrence of the disease could be highly suspected. Complete resection is an effective treatment for hydatid cyst disease. Hydatid cyst disease, also known as echinococcosis, is a parasitic disease that affects humans and other mammals such as sheep, dogs, mice, and horses, relatively common in livestock areas. Humans may become infected by direct contact with the definitive host (dogs) or by ingestion of food infected with the parasite's eggs . In clinA 42-year-old Kazakh patient was admitted to the hospital with pain in his right lower back and weakness in both lower limbs with no inducement for 2 months. The pain was aggravated after activity and was relieved after rest, the Visual Analog Scale (VAS) pain score was 7. Neurological examination revealed bilateral lower extremity weakness: left muscle strength was 3/5, the right was 4/5. With brisk tendon reflexes and negative Babinski sign, the remainder of the physical examination was unremarkable. Besides, the patient grew up in a pastoral area with close contact with cattle, sheep, horses, and other livestock.The CT scan showed the L1 vertebral body and peripheral bone were hyperostotic (smooth edge), the relevant intervertebral were also enlarged. The MRI scan performed an oval solid-cystic cavity at the right side of the vertebral body of the T12~L2 vertebrae and the enlarged L1~L2 intervertebral foramen, the cystic cavity was hypointense on T1-weighted-image (T1WI), hyperintense on T2-weighted-image (T2WI), and T2-fat-suppression-image (T2FS). The multiloculated cystic lesions could be seen inside and had clear boundaries with surrounding tissues. Corresponding intervertebral foramina around the cavity was enlarged, which compressed the conus medullaris of L1 and extended into the adjacent psoas , 2. The We used the posterior straight incision approach of T12~L2. The incision was made to expose the T12~L2. The laminectomy was performed for the right part of the vertebral plate and inferior articular process of the T12, the right vertebral plate and attachment of the L1, and superior articular process at the right side of the L2. Entering the incision, the oval solid-cystic cavity with clear boundaries located at extradural  and had compressed the spinal cord to the left ventral side. The lesion has high tension and tight adhesion to the dura mater and intervertebral foramina. Gauzes were used to protect the incision and the surrounding tissues, we carefully cut the ectocyst to decompress, a whitish, pearly, translucent cystic endocyst was found. After careful separation and loosening, we scratched the edge of the cyst to remove the endocyst as a whole carefully. And loosen the attachment of the dural sac, ensure that it was intact and pulsates well. Then used hydrogen peroxide and 5% hypertonic saline to inject for 10 min, checked again whether there was residual endocyst. Peeled off most of the ectocyst carefully, followed by hydrogen peroxide, 5% hypertonic saline, and dexamethasone irrigated the operative site again to avoid relapse and anaphylactic reaction. Finally, spinal fusion and fixation of T12~L2 have been performed and the wound was closed in layers.In histopathological examination, we found that after Hematoxylin and Eosin (H&E) staining of the tissue, granulomas and inflammatory granulation tissue could be seen, consisting of proliferative fibers, vascular epithelioid cells, and multinucleated giant cells. The hydatid and endocyst's germinal layer could be found . ChronicThe patient recovered well after surgery, the muscle strength of both lower limbs was restored to level 5/5, and the pain was almost completely relieved (VAS score from 7 to 2), Albendazole session was continued for 12 months (10 mg/kg body weight/day) as for standard anti-infectious therapy of Echinococcus. During the follow-up and reexamination for 6 months after surgery, the patients did not show the same similar symptoms, and there was no sign of recurrence on imaging evaluation. We advise the patient to take the medication on time and to review for further imaging every 6 months.Spinal hydatid disease is rare and was rarely reported in the literature. The main infectious source of this disease is livestock, most of which are caused by the invasion of Echinococcosis Granulosa into the human body, and a few are caused by Echinococcosis Multilocularis. The former is the more benign form and is characterized by cyst formation, whereas the latter is not encapsulated and presents as a ramifying, porous, and necrotic mass. The prognosis for the multilocular form is extremely poor, with an almost 100% mortality rate . Spinal In this case, vertebral canal involvement was primary, and no signs of hydatid were found in the vertebrae and other organ systems, the serological test (ELISA) was negative, which made the case extremely rare. In our literature review, Parvaresh et al.  reportedThe early diagnosis of this case for primary hydatid cyst across the intervertebral foramen was difficult, because such cases were extremely rare, and the patient had no hydatid cysts of other organs and no previous history, the serological test was also negative. There were only abnormal MRI signals and significantly enhanced septum of paravertebral and intervertebral foramina. Therefore, it is easy to be misdiagnosed. We collected the detailed data of all published cases of primary extradural intraspinal together with paravertebral hydatid cyst in The diagnosis of hydatid disease depends on radiology, biology, and serology examinations. Serological diagnosis is usually performed by Enzyme-Linked Immunosorbent Assay (ELISA), Western Blotting, Indirect Hemagglutination Assay (IHA), and Polymerase Chain Reaction (PCR). The sensitivity rates of serological diagnosis of the liver, lungs, and other organs are 80–100%, 50–56%, and 25–56%, respectively . In our Surgical removal of the lesion is the best choice for the treatment of spinal hydatid disease, especially for the treatment of patients with spinal cord compression symptoms, surgical removal of the lesion is still the “gold standard.” For the operative approach selection, the lesion is usually deep in the side of the spinal bypass incision approach, which makes it difficult to quickly enter the lesion area and may cause retroperitoneal spread during the operation. Therefore, we need to choose the posterior straight incision approach of the affected spine segment, which can quickly enter the corresponding lesion area and facilitate the complete resection of the lesion. And it is also more convenient for spinal fusion and fixation. For the resection, the removal of hydatid cysts must be thorough, as much as possible to remove all endocyst in the field of surgery. During the operation, spinal cord injury must be avoided, and care should be given to prevent the puncturing of the endocyst wall. Finally, spinal fusion and fixation should be performed to restore spine stability while removing the lesion. Because the damage of hydatid to the spinal cord is mainly mechanical compression, rather than invasive damage. So, if it can be early detection and early surgical removal, the prognosis will be better.In our case, the lesions occurred in the extradural, and the intraspinal lesions were small, with a clear boundary between the internal and external spinal canal and relatively limited adhesion around the surrounding tissues. So, the capsule can be completely removed. Remove the ectocyst completely and avoid residuum of the endocyst, then the disease could be cured. Besides, due to the slow onset of this patient, the large volume of the lower lumbar spinal canal, and it has a certain buffering effect, hydatid cysts extending along the nerve root sheath to the outside of the spine also play a certain role in decompression. And through the fusion fixation of the spine, the patient can do off-bed activity earlier. Therefore, the neurological function recovered well in this case after the operation.Due to the limited space in the spinal canal, if the lesion in the spinal canal is large or at a high position, to avoid excessive compression of the spinal cord during intraoperative operation, the ectocyst can be cut and gauze can be used to protect the surrounding tissues, suction out the fluid of endocyst to decompression, and remove the ectocyst in a whole. If the endocyst ruptures during the operation, the rate of recurrence will be higher. Therefore, it is necessary to avoid intraoperative contamination as much as possible to prevent the hydatid cyst from rupturing and causing it to be planted in the surrounding tissues. Anti-hydatid drugs and appropriate hormones should be used as adjuvant therapy before and after surgery to avoid the occurrence of anaphylactic shock. After the removal of the lesions, it should be washed with hydrogen peroxide and 5% hypertonic saline to prevent recurrence or reduce the recurrence rate, then use dexamethasone water to avoid serious allergic reactions. All these measures are used to avoid various symptoms caused by allergic reactions or overflow of the content of the hydatid head section, such as itching, urticaria, edema, dyspnea, asthma, vomiting, diarrhea, colic abdominal pain, and even anaphylactic shock.Hydatid cyst across the intervertebral foramen has been reported only sporadically in the literature, and the primary cyst is extremely rare. For diagnosis, hydatid cyst disease should be considered when imaging findings indicate cystic changes in the spinal region and the patient has a pastoral life history, even if the serology test is negative. For treatment, the main method is still surgical treatment, and great attention should be paid to completely remove the cyst to avoid extravasation of the cystic fluid to cause spread.As the incidence of this disease is extremely low, rarely encountered in clinical practice, so this case would be reported in the hope of improving the understanding, diagnose it and treat it in time.The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.YT and MJ performed the image data analyses and wrote the manuscript. XS contributed to perform the operation and give the manuscript preparation. YH helped perform the analysis with constructive discussions. LJ helped for giving the literature review. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Gastrointestinal stromal tumors (GISTs) generally form well-defined mass lesions. However, some cases of the flatly distributed and muscularis propria-replacing GISTs have been reported so far. We experienced an additional case of planar-type GIST of the sigmoid colon accompanied by a diverticulum with perforation.kit gene revealed that the lesion had a heterozygous deletion of 2 amino acids at codons 557 and 558 of exon 11. The mutation was not observed in the normal mucosa of the surrounding tissue.A 68-year-old Japanese male with sudden onset of abdominal pain was clinically diagnosed with gastrointestinal perforation, and an emergency abdominal operation was performed. A diverticulum with rupture was found in the sigmoid colon, but no apparent tumor was observed. Histological examination revealed bland spindle cells flatly proliferating and diffusely replacing the muscularis propria at the diverticular structure. The spindle cells were positive for KIT, DOG1, and CD34. Mutational analysis of the c-We diagnosed this case as an unusual planar-type GIST. Some similar cases have been reported in the sigmoid colon and other sites. We discuss the mechanism of development of the planar-type GISTs associated with the diverticulum. GISTs are currently thought to be derived from the interstitial cells of Cajal (ICCs), the GI pacemaker cells . Most GIutations . These KGISTs generally form well-defined mass lesions. However, some cases of the flatly distributed and muscularis propria-replacing GISTs have been reported so far –7. Here A 68-year-old Japanese male with sudden onset of abdominal pain was transferred to Nomura-Kaihin Hospital. He did not have any special past medical history and had never received colonoscopy. Contrast-enhanced computed tomography revealed a locally dilated sigmoid colon with fecaloma and perforation. Findings of feculent peritonitis (stage IV by Hinchey Classification) and free air in the peritoneal cavity were also detected, but no apparent tumor was pointed out. He was clinically diagnosed with perforation of the sigmoid colon, and an emergency operation was performed. During surgery, a diverticulum with wide frontage and rupture was found in the sigmoid colon, but no apparent tumor was observed. Fecaloma was present near the rupture site. The sigmoid colon measuring 16 cm in length including the perforated area was resected. The diverticulum with rupture was present at the contramesenteric side and was 55 × 35 mm in size  patients [kit gene mutations. Thus, this patient does not seem to be a case of NF1. We concluded that this is a case of sporadic planar-type GIST.Most of the sporadic GISTs form nodules of various sizes. On the other hand, patients with germline mutation of the c-ntestine . The flantestine . In the patients , but theFlatly proliferating ICC lesions in the abovementioned two syndromic settings are usually present as thin layers of spindle cell proliferation along the myenteric plexus occupying the intermuscular layer between the inner circular layer and outer longitudinal muscle layer. In contrast, the entire thickness of the muscularis propria was involved in the flat proliferation of spindle cells in the present case. Thus, the morphological characteristic as well as the genetic abnormality is also different between the present case and the syndromic cases.Eight similar cases of flatly proliferating and muscularis propria-replacing GISTs including our case have been reported so far as shown in Table The planar-type GIST in this case was present associated with a true diverticular structure. The diverticula are observed in 3 of 5 reported cases of the sigmoid colon and 3 of 3 reported cases of the small intestine. The planar-type GISTs are considered to be closely related to the diverticular structure. Initial lesions of the planar-type GISTs probably occur within the muscularis propria layer like those of usual GISTs. The initial lesion of any types of GISTs might locally alter gut motility and induce disturbed peristalsis. In the case of the sigmoid colon with the narrowest lumen among the various GI sites, the abnormal bowel movement due to the initial lesion might result in stool accumulation and fecaloma formation. The fecaloma might compress the sigmoid colon wall to the outside and locally extend the lumen of the sigmoid colon. Consequently, the dilated diverticular structure might be formed, and it could be finally perforated by the fecaloma compression. The initial lesion of the GIST might be flattened by the oppression of the fecaloma described above, and the oppression itself might stimulate the growth of the initial GIST lesion sideways followed by development of the flatly proliferating GIST. Thus, the sigmoid colon with the narrowest lumen might have the optimal environment for development of the planar-type GISTs.Kawanowa et al.  reportedRectal GISTs are considered to be more aggressive than gastric GISTs. On the other hand, all of the reported cases of planar-type GISTs including this case showed low mitotic figures and low Ki-67 positive rate, and none of the patients had recurrence of GISTs even in the cases with perforation Table  2, 4, 55, 7. ThuWe described a case of the planar-type GISTs with a diverticular structure. How the planar-type GISTs develop is not clarified, but we should carefully examine whether the diverticula of the sigmoid colon in the elderly patients have planar-type GISTs.


Hydatid diseases are a cystic parasitic infestation caused by Echinococci which are endemic to parts of India and commonly occur in agricultural workers. Primary intramuscular hydatidosis is a rare manifestation of hydatid disease.Our case report describes a rare case of primary intramuscular hydatidosis in an agricultural worker with no involvement of any other viscera. This was diagnosed with clinical evaluation, radiological modalities and managed surgically.Hydatid disease is endemic in parts of India. Primary intramuscular hydatidosis is uncommon because the lactic acid in the muscle and muscle contractility hinders the development of cysts making it improbable diagnosis on first presentation. Hydatid cyst demonstrates a wide variety of imaging features, which can vary according to growth stage, associated complications and affected tissue. The treatment of choice is complete surgical excision of the cyst along with thorough irrigation of the surrounding soft tissues combined with the use of systemic antiparasitic drugs after surgery.In a patient of an agricultural background of cattle rearing living in an endemic region and presenting with a swelling in the musculoskeletal system, a suspicion of hydatidosis should always be kept in the clinicians mind, so that it can be diagnosed using a multimodal approach and managed properly in a timely manner. These are commonly encountered in farm workers dealing with agriculture and livestock . The org2A 52-year-old male working as a cattle rearer in a farm in rural India, presented with complaints of swelling over left thigh region. Disease onset was 2 years ago when patient first noticed a small swelling over left thigh. Swelling was insidious in onset, spontaneous in origin and gradually progressed to a size of 18 × 12 cm. There was no period of rapid increase or regression in size of swelling. There was no history of pain over swelling. There was no history of trauma or fever. No history of any comorbidities. General examination of the patient showed no irregularities. Physical examination showed a firm, non-tender mass of the upper two third of the left thigh. Overlying skin was normal. No other visible or palpable swelling was present in the rest of the body. All routine blood investigations were normal. Ultrasonography (USG) revealed large multi-loculated complex cystic mass measuring 31 × 29 × 19 cm (volume 236 mL) in the anterior and medial aspect of the upper two third of left thigh predominantly in subcutaneous plane. Magnetic Resonance Imaging (MRI) revealed well encapsulated cystic lesions comprising of multiple daughter cysts of varying sizes within them, involving the anterior and posterior compartments of left thigh  measuring approx. 17.2 cm (SI) × 10.6 cm (TR) × 8.7 cm (AP) and 25 cm (SI) × 17 cm (TR) × 7 cm (AP) respectively. The lesions showed mild peripheral enhancement on post contrast study and were seen causing compression over the adjacent muscles and anterior neurovascular bundle. No skeletal involvement was seen .Fig. 1MRChest X-ray and Contrast Enhanced Computed Tomography (CECT) abdomen pelvis and chest did not reveal any other organ involvement. On the basis of these findings a diagnosis of intramuscular hydatid cyst of the left thigh was established and surgical excision was planned. Post-operative histopathological examination confirmed our diagnosis of intramuscular hydatid cyst.3Under spinal anesthesia, 2 incisions were given over left thigh. Vertical incision anterolaterally (9 cm) and oblique incision medially (13 cm). 3 large cysts were located in the anterolateral, anteromedial and posteromedial compartments. The anteromedial and posteromedial cysts were found to be communicating. Posteromedial cyst extended upto gluteal region. After securing the wound with sponge soaked in hypertonic saline (3%), the cyst was opened up and upto 2 L of dirty putrefied material along with daughter cysts were removed. Pericystectomy was done for all cysts along with evacuation of daughter cysts. Thorough wash was given with 10 % povidone iodine. Two abdominal drains (28Fr) were inserted in the anteromedial and posteromedial compartments and the wound was closed primarily. Post-operative period was uneventful. Both drains were removed on the second day. Patient was discharged on the third day .Fig. 2(a4On discharge, patient was advised albendazole therapy for one month. Follow up was advised at 15 days, 1 month, and 3 months. Patient had no local or systemic complications. There were no clinical signs of recurrence. USG was repeated on each follow up and showed no residual cysts or recurrence.5Hydatid disease is an endemic zoonosis caused by Echinococcus granulosus prevalent mainly in countries where sheep raising is practiced; however, due to increased travel and tourism around the world, it can be found anywhere, even in developed countries. In India, hydatid disease is commonly seen in the southern states of Andhra Pradesh and Tamil Nadu .Hydatid disease can be primary or secondary. Primary hydatid disease is spread by the ingestion of the ova and may develop in almost any organ. Liver and lungs are the most frequently affected organs. In secondary hydatid disease, larval tissue proliferates after spreading from the primary site. However, there is a primary site of hydatid disease situated in the liver, lung, or spleen .Hydatid cyst located primarily in the intramuscular tissue is an extremely rare manifestation of the hydatid disease. Patients usually present with a painless, slow growing mass with normal overlying skin . PrimaryThe pathogenesis of muscular localization remains a matter of debate. Some authors claim that it is because of direct implantation through a wound, for instance, through the bite of a dog. However, most authors believe that the embryo reaches the muscles via systemic circulation after leaving the intestine and passing through the liver and the lungs which act as filters . Soft tiOther possible primary locations of the disease must be ruled out by careful clinical and radiological examination of the patient. The Casoni skin test and various serological tests are available for the diagnosis of hydatidosis. They may help to distinguish hydatid cyst from other non-parasitic cysts and abscesses but can give negative results in case of encapsulation of the lesion. Thus, a negative test does not rule out the possibility of hydatid cyst .USG, CT, and MRI are used to describe the location and features of the cyst. Hydatid cyst can demonstrate a wide variety of imaging features, which can vary according to growth stage, associated complications, and affected tissue. The radiologic findings can range from purely cystic lesions to a completely solid appearance .USG is a good diagnostic tool and has a high diagnostic accuracy. USG is particularly useful for the detection of cystic membranes, septa and hydatid sand, floating membranes, daughter cysts and vesicles. CT is a useful modality to demonstrate cyst wall calcification, the internal cystic structure posterior to calcification and cyst infection . HoweverBased on typical imaging findings of a multilocular encapsulating cyst with multiple daughter cysts along with mild peripheral enhancement on post contrast study, a diagnosis of intramuscular hydatidosis was made preoperatively in our case.It is crucial to identify or suspect the presence of a hydatid cyst before surgery, or else the cyst’s contents may be ruptured and may spill into the systemic circulation as a result of improper handling during surgical resection. This may induce an anaphylactic reaction as discussed previously ,15. The 6Primary intramuscular hydatidosis is an extremely rare manifestation of Hydatid disease which is endemic in India. The diagnosis of this disease proves challenging and requires a multimodal approach. The patient presented to us with a large swelling over his left thigh and a diagnosis was established with detailed history, clinical examination along with radioimaging. The patient was treated with pericystectomy for all the cysts along with evacuation of daughter cysts followed by albendazole therapy.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.No ethical clearance was needed for this case report.Written informed consent was obtained from the patient for publication of this case report and the accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Dr Vaibhav Lakhanpal: Writing the paper.Dr Mohit Badgurjar: Operated, revised and edited manuscript.Dr Pankaj Saxena: Operated.This was not a first in man study.Dr Mohit Badgurjar.Not commissioned, externally peer-reviewed.I am thankful to the team of surgeons for accurately detecting my problem, operating on it and treating me so I could rejoin my work with full vigour.The authors report no declarations of interest.


Hybrid lesions encompass the occurrence of different entities in one lesion. A 67-year-old woman was referred to the Oral and Maxillofacial Surgery Service for treatment of mandibular Central Giant Cell Lesion (CGCL) previously diagnosed. Intraoral examination revealed edentulism and a painless swelling extending from the alveolar ridge to the buccal vestibule with hard consistency covered by normal mucosae, with unknown duration. Panoramic radiograph revealed a large, multilocular and well-defined radiolucent lesion extending from the region of left mandibular lateral incisor teeth to right mandibular first molar with no evidence of osseous perforation. Initially, a treatment with intralesional injection of corticosteroids was performed. After 18 months of treatment, an increase in size of the osteolytic lesion was noted. An incisional biopsy was carried out and the microscopic examination revealed a unicystic ameloblastoma associated to CGCL. It was performed marsupialization and later the enucleation of residual lesion. The follow-up remains being performed. Key words:Hybrid lesion, central giant cell lesion, ameloblastoma. Hybrid lesions encompass elements of different pathologies in one lesion and the occurrence within the jaws are rarely reported . Among tCentral giant cell lesion (CGCL) is a benign lesion of the jaws with an unknown etiology. These lesions occur more frequently in females until third decade of life and are often located on the anterior region of mandible. Most cases presents as a intraoral slow growing and painless swelling. The radiological features are diverse and may vary from small unilocular to extensive multilocular radiolucent areas, beside displacement of teeth and tooth germs, root resorption, and cortical perforation. Histopathologically, CGCL is characterized by a cellular connective tissue permeated by mesenchymal ovoid and multinucleated giant cells of various sizes and, occasionally, with multiple foci of hemorrhage and trabecular bone tissue .et al. classified aggressive and non-aggressive lesions according to signs and symptoms and histological features. The lesion is considered aggressive when several of the following features are present such as pain, paresthesia, root resorption, rapid growth, cortical perforation, and high recurrence rate. On the other hand, the non-aggressive lesion is characterized by slower growth and absence of cortical perforation or tooth resorption  applied intralesionally. To reassess the response to the treatment, panoramic radiographs were performed in Dec/2016  stained sections showed a combined epithelial and mesenchymal lesion. The first component revealed an odontogenic epithelial lesion predominantly cystic, but with solid areas, exhibiting epithelium with palisaded basal cells with hyperchromatic nuclei, focal reverse polarization and upper layers with stellate-reticulum-like cells Fig. a,c. The After that, it was performed marsupialization on cystic component in order to promote bone formation decreasing the cystic cavity. After three months, the whole lesion was removed by enucleation with peripheral osteotomy. Once again, the specimen was submitted to microscopic examination and the diagnosis of UA associated to CGCL was confirmed. Immunohistochemistry against CD68 highlighted the multinucleated giant and the mesenchymal cells Fig. d, whereaThe last radiography was taken in May 2019  , fibro-oet al.  a collision tumor, which results from the synchronous occurrence of the lesions; b) COF is the primary lesion and induces a GCGL reaction in response to trauma or other stimulus; or (c) CGCL produces growth factors, chemokines and cytokines that stimulates the proliferation of odontogenic cells, resulting on the COF .Since our case is the first one described in the literature, there are no previous studies about its pathogenesis. Based on theories described above, clinical history and radiographic examinations, this case seems to be a real collision tumor for two majors reasons: first, the primary incisional biopsy revealed just the CGCL, without signs of odontogenic lesion, leading to the conclusion that CGCL was the predominant lesion in the beginning. The second point is the fact that the patient responded well to the treatment with intralesional corticosteroids, a specific treatment against CGCL, for 18 months. Therefore the patient probably had two separated lesions that collided when the UA growed up and reached the CGCL. In addition, we forwent the theory that the CGCL would stimulate the proliferation of odontogenic cells, because if this was a typical phenomenon, we believe that the presence of odontogenic tumors associated with CGCL would be commonly seen.Currently, there are few cases of collision tumors published, probably because these lesions are unnoticed and/or diagnosed according to one of the prominent microscopic features. This limited number of cases lead to be difficult to predict their biologic behavior .et al. was the first to report the use of corticosteroids in the treatment of CGCL. In their case it was administered systemic dexamethasone, which leads to lesion reduction but also to systemic complications (In the presented case, the initial diagnosis was CGCL. Due to lesion size and patient’s age, initially a conservative treatment was carried out. The most common intralesional drug used for CGCL in the cases reported in the literature is triamcinolone acetonide , but dexications . In 1994Due to the good results previously described with the use of dexamethasone, this modality of treatment was applied in the current patient. With the last biopsy revealing the presence of a UA component, surgical approach was planned. The marsupialization was performed followed by enucleation of the residual lesion with peripheral osteotomy to prevent recurrence.Some studies have reported a higher risk of recurrence for hybrid lesions or collision tumors with CGCL component, recommending a long-term follow up and careful management . After 1To the best of our knowledge, this is the first case of UA and CGCL occurring simultaneously reported in English literature. Despite the clinical, radiological and histopathological features, the exact timing of onset of the lesions, concomitantly or independently, remains uncertain. It is necessary further studies with long-term follow-up information to understand the pathogenesis and biologic behavior of hybrid lesions and collision tumors.


Primary hyperparathyroidism may present in a myriad of manners, varying from an incidental asymptomatic biochemical finding to gastrointestinal, psychiatric, renal, and bone manifestations. While hyperparathyroidism remains a rare diagnosis in the pediatric population, the initial approach to diagnosis and management of hypercalcemia in children is imperative for the general pediatrician. Herein, we describe an adolescent who presented with a lytic bone lesion and severe, symptomatic hypercalcemia due to primary hyperparathyroidism.A 14-year-old male presented with vomiting, constipation, abdominal pain, and lethargy. He had an elevated total corrected calcium of 4.3 mmol/L. He was found to have a large pelvic lytic tumor consistent with a brown tumor due to primary hyperparathyroidism. He received pharmacologic therapy for stabilization of his hypercalcemia, including intravenous saline, intravenous bisphosphonates, and calcitonin. He subsequently received definitive therapy via parathyroidectomy and his post-operative course was complicated by hungry bone syndrome. Long-term follow-up has found full resolution of the lytic lesion and restored calcium homeostasis. We present this case to highlight the possible presentations of hypercalcemia and hyperparathyroidism that are essential for a general pediatrician to recognize to ensure prompt diagnosis and management. Evaluation for hypercalcemia should be considered in patients with suggestive symptoms and physical exam findings. To our knowledge, this patient represents the first reported pediatric case of a pelvic brown tumor in an adolescent. While the multi-systemic complications of hyperparathyroidism may be quite severe, swift and appropriate management may mitigate these clinical outcomes. The presentation is commonly an incidental biochemical finding with asymptomatic hypercalcemia. If symptomatic, patients may describe fatigue, headache, nausea, abdominal pain, vomiting and polydipsia. End organ complications are also described, including nephrocalcinosis, renal impairment and osteoporosis. Bone manestation. We reporA 14-year-old previously healthy male was admitted with a 3-week history of daily emesis, constipation, and lethargy. He had a decreased appetite and 3 kg weight loss in the preceding weeks. He described vague periumbilical pain and his family noticed a recent depressive change in his mood, including a fatigued appearance, low mood, and withdrawn behaviour. He had no joint or bone pain and denied fever or night sweats. Past medical history was unremarkable. He was on no medications. The family had recently immigrated from the Philippines one-week prior to the onset of symptoms. On exam, he appeared unwell and cachectic with a weight of 37 kg (-2.1 standard deviation (S.D.)) and a body mass index (BMI) of 14.1 kg/m² (<-3 S.D.). He was alert, oriented and cooperative. Neurological exam was unremarkable with normal deep tendon reflexes, bilateral flexor plantar reflexes, a normal gait and normal strength. A large, irregular, tender, mobile abdominal mass of 10 × 5 cm was palpable in the left lower quadrant.Initial laboratory testing demonstrated severe hypercalcemia and hypophosphatemia, with a corrected total serum calcium of 4.30 mmol/L (N: 2.14–2.65 mmol/L) and a phosphate of 0.58 mmol/L (N: 0.74–1.52 mmol/L). Initial serum intact parathyroid hormone (PTH) was elevated at 51.70 pmol/L (N: 1.30–6.80 pmol/L), later peaking at 70.2 pmol/L. Additional biochemical evaluation on presentation is provided in Table Given the palpable mass and reported symptoms of emesis and constipation, a plain film abdominal radiograph was performed on admission to evaluate the abdominal mass and assess for possible obstruction. This identified large fecal loading of the colon and a lytic lesion within the left iliac wing Fig. . SubsequNo further bony lesions were identified with full body skeletal survey radiographs and scintigraphy. Renal ultrasound was unremarkable with no nephrocalcinosis nor nephrolithiasis. Bone mineral densitometry (BMD) demonstrated lumbar spine, whole body and left distal forearm Z-scores of -0.2, -1.1, and − 1.7, respectively.CT guided core needle biopsy of the iliac bone lesion was performed and underwent histological assessment demonstrating viable bone surrounded by moderately cellular spindle cells with hemosiderin, hemorrhage, and rare osteoclast-like giant cells, consistent with osteitis fibrosa cystica Fig. .Fig. 4C). The postoperative course was complicated by prolonged, recurrent hypocalcaemia with a total serum calcium nadir of 1.98 mmol/L, consistent with “hungry bone syndrome”. He required IV and oral replacement of calcium for 6 days. He was later discharged to home with oral supplementation of calcium carbonate and cholecalciferol.Prior to surgical treatment, hypercalcemia was treated with intravenous (IV) normal saline, multiple doses of subcutaneous (SC) calcitonin and a single dose of IV Zoledronic acid 0.025 mg/kg). The patient underwent surgical excision of a left superior parathyroid gland  with histology consistent with a parathyroid adenoma  and BMI to 19.8 kg/m² (0.0 S.D.). Serum biochemistry was maintained with gradual weaning and discontinuation of supplementation of calcium and vitamin D; serum total calcium 2.43 mmol/L, phosphate 1.66 mmol/L, and PTH 6.2 pmol/L. Repeat pelvic MRI within 12-months post-parathyroidectomy demonstrated complete resolution of pelvic lesion.Hypercalcemia is a relatively rare presentation in the pediatric population, however, an approach to the differential diagnosis and awareness of initial management steps is important for the general pediatrician. While hypercalcemia in neonates and infants includes a number of genetic and iatrogenic possibilities, the diagnosis in the adolescent – much like in adults – is most commonly due to one of three aetiologies: hyperparathyroidism, malignancy or immobilization. Given thInitial management for all patients with severe symptomatic hypercalcemia must begin with IV fluid administration for dilution and subsequent renal excretion of excess calcium. Hypercalcemia can induce dehydration through impaired oral intake and is compounded by possible development of nephrogenic diabetes insipidus. Additional therapies to reduce serum calcium levels may include antiresorptive agents (i.e. bisphosphonates), calcitonin, forced calciuresis, and corticosteroids. Bisphosphonates inhibit osteoclastic activity and hinder hydroxyapatite breakdown, a mineral composed of calcium and phosphate. Calcitonin inhibits bone resorption and forces renal excretion of calcium, but its effect is limited due to development of tachyphylaxis within days of initial exposure. Facilitating calciuresis through diuretic use, such as furosemide, should be delayed until intravascular volume has been replaced. The use of corticosteroids is potentially beneficial in select etiologies of hypercalcemia that are primarily mediated by excessive production of activated vitamin D (ex. granulomatous diseases).This patient highlights several unique aspects to the presentation of hyperparathyroidism. Primary hyperparathyroidism is characterized by the excessive secretion of PTH by one or multiple parathyroid glands. PTH promotes bone catabolism of cortical bone and bone anabolism of cancellous bone. This wasThe diagnosis of a brown tumor is primarily clinical through the presence of either a single or multiple well-demarcated osteolytic lesions in the setting of hyperparathyroidism and hypercalcemia. Histologic features of the tumor are non-specific and may resemble other giant cell tumors , 7 and iDefinitive therapy for primary hyperparathyroidism and brown tumors is surgical through parathyroidectomy. In cases of severe, prolonged hyperparathyroidism, there is a significant postoperative risk of developing hungry-bone syndrome. This resWith a wide spectrum of presenting features, severe hyperparathyroidism may result in significant pathology, multisystemic involvement with end-organ damage requiring urgent recognition and therapy. Its mimicry of malignancy requires a thorough clinical review and for patients presenting with severe disease, a combination of acute pharmacologic treatment to reduce hypercalcemia followed by definitive surgical intervention are required for optimization of short- and long-term outcomes. This case reports an extremely rare manifestation of chronic hyperparathyroidism, pelvic brown tumor, and exemplifies the importance of clinical recognition of hypercalcemic symptoms to facilitate prompt diagnosis and acute management.


This kind of tumor is usually well-differentiated with low to no potential to metastasize. Thymoliposarcomas are extremely rare tumors that are difficult to diagnose and differentiate from thymomas and other benign conditionsPresentation of a case:This report presents a case of a 46-year-old male patient with dyspnea, generalized fatigue and non-specific chest pain caused by a giant anterior mediastinal mass. Computed tomography scan revealed a large mass in the anterior mediastinum. CT guided biopsy was consistent with thymolipoma. The tumor was surgically resected. The histological analysis of the tumor revealed thymoliposarcoma.Discussion:Thymoliposarcoma usually presents with non-specific symptoms. The mean age of the diagnosis is 55.8 years old with a slight predominance in males. The corner stone of the treatment remains surgical excision of the tumor while the role of adjuvant therapy is not well documented. •Thymoliposarcoma is an extremely rare diagnosis.•The mean age of diagnosis is 55.8 years with a slight predominance in males.•The mainstay of treatment remains surgical resection of the tumor.•The role of adjuvant therapy is not well documented in the literature. The diagnosis of thymoliposarcoma can be challenging, as it is hard to be distinguished from other thymus or anterior mediastinal tumors on imaging. Complete surgical resection is the standard treatment; the role of adjuvant therapy is not well established [Liposarcoma is most frequently seen in the retroperitoneum; however, it can also develop in unusual sites ,2. Thymoablished .The presented case in this report is the largest thymoliposarcoma in the literature. The rarity of this tumor brings the importance to such cases to understand its presentation, clinical course and best available treatments. Furthermore, this review represents the first review in the literature of thymoliposarcoma. We believe that this review will help future doctors and researches to conduct further studies in this field.2In addition to the presented case, MEDLINE database for all published articles of thymoliposarcoma in the literature is also reviewed. Only liposarcoma involving the thymus gland were included. There was no restriction on language, country or publication date of the paper published. This work is reported in line with SCARE 2020 criteria .3A 46-year-old male with no significant medical, surgical, psychosocial or family history presented to our institution with a yearlong complaints of fatigue, shortness of breath and non-specific chest pain see . Part of4Thymoliposarcoma is a very rare mesenchymal tumor that can reach a large size at diagnosis and remains asymptomatic . The tumLess than 200 cases of primary mediastinal liposarcoma have been reported so far; however, only 10 previous cases involved the thymus and were diagnosed as thymoliposarcomas . This caNone.None.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Not commissioned, externally peer reviewed.The first author is the guarantor of this article


Primary melanocytic tumors of central nerve system (CNS) are rare, primary diffuse leptomeningeal melanomatosis (PDLM), a subtype of malignant melanomas of CNS, is extremely rare,especially in pediatrics. As the clinical manifestation of PDLM is not characteristic, It is often misdiagnosed as tubercular meningitis and hemorrhage.A 13-year-old boy was admitted to our department with symptoms of recurrent headache and vomiting twice. As the brain imaging revealed a lesion located in the left temporal lobe mimicked hemorrhage, so there was a misdiagnosis of hemorrhage in first hospitalization. He was admitted again for the recurrence of the headache and vomiting. Detailed physical examination showed multiple melanin changes in the skin of the whole body which were ignored in last hospitalization. Brain imaging showed the significantly enlarged lesion in the left temporal lobe and several smaller lesions in the left parietal lobe and cerebellum which indicated metastasis.According to the history,physical examination and the radiological finding, the patient was diagnosed with malignant melanoma of central never system possibly.The patient underwent left temporal and parietal lesions total resection with a craniotomy.The diagnosis of PDLM was established according to pathological characteristics and the negative finding of positron emission tomography (PET)-computed tomography (CT) outside CNS. The patient got no further treatment for economic reasons and experienced the progression and died 5 months after operation.PDLM is extremely rare in CNS, as the clinical manifestation, radiological changes are not special, early diagnosis is difficult. The confirmed diagnosis is established by leptomeningeal biospy or surgical tissue. PET-CT can help differential diagnosis with metastastic leptomeningeal melanomas. The prognosis is dismal due to the inefficiency of chemotherapy or radiotherapy. PDLM is a rare subtype of malignant melanomas of the CNS, with an incidence of 1 case per 10 million individuals. As the incidence is extremely low and the clinical manifestation of PDLM is not characteristic, it is often misdiagnosed as tubercular meningitis and hemorrhage. To the best of our knowledge, only 32 cases of PDLM have ever been reported in the literature, and only 4 of these were pediatric cases. Here we present a rare case of pediatric PDLM mimicking hemorrhage, which represents the very first pediatric PDLM case in China. In addition, we performed a comprehensive review of the related literature.Melanocytic neoplasms of the central nervous system (CNS) can be divided into primary neoplasms and metastasis. Importantly, primary melanocytic tumors are rare, and just account for only 1% of all melanomas and 0.05% of primary brain tumors.21-WI and isosignals on T2-WI, and with significant enhancement after administration of gadolinium  scan revealed a high-density lesion in the left temporal lobe, suggesting hemorrhage  absorption. However, focal neurological deficits are rare. Considering that tumor apoplexy is common, the misdiagnosis of cerebral hemorrhage usually happens in the early stage such as in our case. Magnetic resonance angiography (MRA) or DSA can help eliminate bleeding due to vascular diseases. It has been reported that the proportion of patients with a large congenital nevus combined with intracranial malignant melanoma is about 25%. Hence, additional brain imaging must be performed to rule out the possibility of malignant melanoma if a patient suffers from increased intracranial pressure in combination with a large congenital melanocytic nevus.The clinical manifestations of PDLM are nonspecific, mainly including headache, vomiting, and seizures, which often mimic the diagnosis of meningitis.1-WI, iso-signals on T2-WI, and obvious enhancements after the injection of gadolinium. MRI enhanced scans may therefore help the diagnosis of this rare disease. Similarly, in the case reported here, the misdiagnosis was made based on imaging findings. Considering the vague presentation of symptoms and radiographic appearance of PDLM, a differential diagnosis should be considered in such cases. Considerations can include but are not limited to tubercular meningitis, hemorrhage, and metastatic melanomas. It has been reported that cerebral spinal fluid (CSF) analysis to confirm the pleomorphic cells with intracellular melanin and a high mitotic index can help an early diagnosis, although the positive rate is extremely low. PET-CT can support a differential diagnosis with metastastic leptomeningeal melanomas, as our case suggests.Imaging of PDLM also shows a lack of characteristic changes. Brain CT scans usually show a circular lesion with high-density mimicking hemorrhage. MRI scans may provide more diagnostic information of this rare disease. Due to the paramagnetic nature of melanin, typical MRI findings of PDLM may be high signals on T Identification requires immunohistochemic examination. The gross specimens of PDLM are mostly solid, growing on the surface of the brain, and are in close relation with the leptomeninges, with or without nodule formation. Intratumoral hemorrhage is common due to the rapid growth. Under the microscope, mixed epithelioid, and spindle tumor cells can be identified, displaying hyperchromatic nuclei. The melanin particles are visible, and the mitosis and abnormality are obvious. Immunohistochemical examinations show S-100 (+), HMB-45 (+), Melan A (+), and EMA (-); these changes are in line with the pathology observed in our case. PET-CT showed no changes outside the brain, and PDLM was thus diagnosed pathologically in combination with the observed clinical manifestations.The confirmed diagnosis of PDLM depends on pathological examination, either leptomeningeal or surgical tissue biopsy. However, there is no significant difference to the general and histological changes observed in other melanocytic diseases. The cause of death is the progressive neurological deterioration and intractable intracranial hypertension. Recently, new molecular targeted drugs, such as the BRAF inhibitor vemurafenib and the antiprogrammed cell death-1 antibody nivolumab, have been reported to be effective against previously untreated metastatic melanoma.,14 These studies show the potential for vemurafenib and nivolumab in the treatment of brain metastasis. The first clinical application of vemurafenib and nivolumab for PDLM has been reported by Fujimori and colleagues; however, whether this approach is useful for improving the prognosis needs to be assessed in a further clinical trial.As PDLM is neither sensitive to chemotherapy nor radiotherapy, the prognosis is extremely poor, and the majority of patients die between 1 month and 2 years after onset, with an median survival time of 7 months.Herein, we present an extremely rare case of PDLM in a pediatric patient. The clinical manifestation and radiographic changes are not specific, and an early diagnosis is therefore difficult. The confirmed diagnosis is established on the basis of leptomeningeal or surgical tissue biopsy. PET-CT can help the differential diagnosis by identifying metastastic leptomeningeal melanomas. The prognosis is however dismal due to the inefficiency of chemotherapy and radiotherapy. To the best of our knowledge, this is the first pediatric case of PDLM in China.Conceptualization: Xinke XuData curation: Xinke Xu, Yaqi ZhengResources: Fenghua WangInvestigation: Junliang LiWriting – original draft: Xinke XuWriting – review and editing: Xinke Xu, Fangcheng Li


Intracranial venous hypertension has been associated with a few cases of meningioma secondary to compression of the venous sinus. This is the rare case of small meningioma involving the sigmoid sinus leading to intracranial venous hypertension mimicking venous thrombosis.A 39-year-old woman suffered visual dysfunction due to bilateral papilledema. Noncontrast head computed tomography (CT) showed no intracranial space-occupying lesions or hydrocephalus. Cerebrospinal fluid examination revealed high opening pressure. Various image inspections such as three-dimensional CT angiography, magnetic resonance imaging, and cerebral angiography demonstrated a small 2.5-cm lesion causing subtotal occlusion of the dominant right sigmoid sinus. No improvement of clinical manifestations was observed after medical treatment for 6 months, so right presigmoid craniectomy was performed. Operative findings revealed that the tumor was located predominantly involving the sigmoid sinus. The pathological diagnosis was fibrous meningioma. Postoperative fundoscopic examination showed improvement of bilateral papilledema.We treated a patient presenting with intracranial hypertension due to a small meningioma involving the sigmoid sinus. This unusual case suggests that early surgical strategies should be undertaken to relieve the sinus obstruction. Meningiomas are the most common type of extra-axial tumors of the meninges, and may occur at any location along the meninges encasing the central nervous system. Many meningiomas are commonly asymptomatic and found incidentally, but large meningiomas can manifest as symptoms of mass effect such as headache, focal neurological deficits, and seizures. Intracranial hypertension has been associated with a few cases of meningioma secondary to compression of the venous sinus –3. IntraWe describe a rare case of intracranial hypertension caused by meningioma located inside the dominant sigmoid sinus.2). Her medical and family history was unremarkable, and no evidence of infection was found including sinusitis, otitis media, and mastoid cellulitis. She was not taking any medication, including oral contraceptives or hormonal agents. She had previously experienced four normal vaginal deliveries (gravidity and parity G4P4) without complications. Cerebrospinal fluid (CSF) examination revealed high opening pressure (500 mmH2O [> 35 cm]) on lumbar puncture and normal CSF composition.A 39-year-old woman presented to the ophthalmology department of our hospital with discomfort in her eyes. Fundoscopic examination showed bilateral papilledema. Extensive neuro-ophthalmologic examination revealed mild visual field abnormalities. She was referred to the neurosurgery department for suspected abnormally high intracranial pressure (ICP). Neurological examination found normal consciousness with no abnormalities. Her cranial nerves were intact. She had no diplopia, weakness, ataxia, or sensory disturbance. Coagulation profile and biological tests were within normal limits. She was overweight (body mass index of 27.2 kg/mNoncontrast computed tomography (CT) revealed no intracranial space-occupying lesions or hydrocephalus except an asymptomatic arachnoid cyst of the left middle fossa Fig. a. The clCT venography demonstrated subtotal occlusion of the right sigmoid sinus, caused by a well-defined, homogeneous, hypodense mass. Conventional angiography demonstrated the dominant right transverse sinus with hypoplastic left transverse sinus and subtotal occlusion of the dominant right sigmoid sinus in the venous phase, and a mass lesion causing severe luminal narrowing Fig. e-f. We c2O. We judged that improvement due to medical treatment could not be expected, so we planned surgical treatment.We investigated methods for improving blood flow by intravascular surgery such as stent placement and percutaneous transluminal angioplasty using a balloon. However, preoperative angiography showed antegrade right transverse flow and poor collateral flow indicating this flow is dependent even in subtotal occlusion. Therefore, sacrifice of the transverse or sigmoid sinus seemed to introduce critical risk. Surgical treatment or intravascular surgery may be high risk without knowing specific location of the mass lesion, inside or outside the sinus, and without knowing the pathological type. Therefore, our patient was treated with lumbar puncture followed by acetazolamide. Oral warfarin was initiated because it was unable to exclude the possibility of venous thrombosis. Frequent ophthalmologic assessments including quantitative visual fields were planned. We expected development of collateral flow and improvement of ICP, but unfortunately imaging showed no change. Slight deterioration of the bilateral papilledema was noted 6 months later. Lumbar puncture confirmed persistent elevation of CSF pressure with opening pressure of 420 mmHRight presigmoid craniectomy was performed for tumor resection under motor evoked potential and somatosensory evoked response monitoring. The transverse and sigmoid sinuses were exposed by drilling of the petrosal bones. The transverse and sigmoid sinuses were elastic and hard, suggesting very high pressure in the sinuses Fig. b. IntraoHistological examination of the surgical specimen revealed spindle-shaped tumor cells, with narrow rod-shaped nuclei arranged in intersecting fascicles, without mitotic activity, nuclear atypia, or necrosis. The pathological diagnosis was fibrous meningioma World Health Organization grade I.The patient’s postoperative course was uneventful. Postoperative MR imaging showed that the tumor was totally resected Fig. a. PostopIdiopathic intracranial hypertension (IIH) is a condition associated with increased ICP in the absence of intracranial pathological findings such as mass lesions or cerebral edema . The cauIn the present case, the differential diagnosis after non-contrast CT is venous thrombosis or IIH based on clinical presentation, ophthalmological evaluation for papilledema, and radiological findings of absence of intracranial space-occupying lesions, brain edema, or hydrocephalus. CT venography demonstrated filling defect in the dominant sigmoid sinus indicating venous thrombosis. The MR imaging findings of venous thrombosis are expected to show acute thrombus as isointense on T1-weighted images and hypointense on T2-weighted images, and subacute thrombus as hyperintense on T1- and T2-weighted images. Thrombus appears as prominent hypointense on susceptibility-weighted images  and T2*-Meningioma sometimes invades the dural venous sinus. Meningiomas can be classified according to the degree of sinus invasion : Type I,Surgery is considered to carry high risk if the lesion location is unclear inside or outside the sinus, or if the tumor invades but does not completely obliterate the dominant transverse or sigmoid sinus, and outflow is strongly dependent on this sinus. Sacrifice of the sinus under such conditions can be a fatal complication such as hemorrhagic venous infarction, diffuse cerebral edema, seizures, or even death , 26. TheWe describe a rare case of intracranial venous hypertension due to a small meningioma causing obstruction of the dominant sigmoid sinus. This unusual case suggests that early surgical strategies should be undertaken to relieve the sinus obstruction.


Primary cardiac lymphoma is a rare tumor, especially a tumor located in coronary sinus (CS). The most common symptom of cardiac tumors is dyspnea, accounting for 64%, followed by chest pain, accounting for 26%. However, the cases with paroxysmal supraventricular tachycardia (SVT) as a major clinical presentation are extremely rare.We report a 55-year-old female patient with primary CS lymphoma and paroxysmal SVT.After the surgical resection, pathology revealed the evidence of diffuse large B-cell lymphoma.The patient underwent chemotherapy after CS tumor resection.The patient was disease-free during the 6-month follow-up.CS enlargement may be the cause of SVT. Echocardiography should focus on the CS section to arrive at the right diagnosis. Primary cardiac lymphomas (PCLs) are extremely rare. In addition, 92% of cardiac lymphomas involve the right atrium or right ventricle, whereas only 7% involve the left ventricular system, and only 1 case has been reported involving coronary sinus (CS). The most common symptom of cardiac tumors is dyspnea, accounting for 64%, followed by chest pain, accounting for 26%. However, the cases with supraventricular tachycardia (SVT) as a major clinical presentation are extremely rare. As the survival period of PCL is only 7 months, and most patients die within a couple of months after diagnosis, early diagnosis and treatment of PCL are extremely important. Hereby, we report a case of primary CS lymphoma and review the literature, with focus on the mechanism of its arrhythmia and the significance of imaging investigations for disease diagnosis in clinical practice.Primary cardiac tumors are uncommon, with an autopsy diagnosis rate of about 0.02%; about 25% of cardiac tumors are malignant,29 cells/L, neutrophils (70.4%), and C-reactive protein level (1.7 mg/L). The possibility of infection was excluded. The d-dimer was 128 μg/L, eliminating the possibility of thrombosis. HIV test was negative. Hepatitis B and C and tuberculous testing were negative. No metastasis was detected in other organs by positron emission tomography. Therefore, there was a high likelihood that this mass was a primary malignant tumor, recognized based on TTE and CT imaging features. To prevent cardiac complications, we performed an emergency surgery to remove the tumor. We utilized echocardiography to monitor the surgical process. The mass was ellipsoidal in 3-dimensional transesophageal echocardiography. The boundary of the mass was clear. The vessel wall of CS was not damaged and the surrounding structure was not involved , CD30, Bcl-2, Bcl-6, PAX-5, CyclinD1, c-Myc , CD23 (FDC+) . No complications occurred after the operation, and no abnormality was found on the repeat ECG. Bone marrow biopsy demonstrated no evidence of lymphoma involvement. The disease was considered to be an extranodal marginal zone lymphoma of Ann Arbor stage IE. The patient subsequently received chemotherapy. Six cycles of chemotherapy with rituximab, cyclophosphamide, epirubicin, vincristine, and prednisone (R-CHOP) were completed. Thereafter, the patient was followed up for 6 months after the last cycle of R-CHOP and found to be completely asymptomatic.A 55-year-old woman presented with palpitations of a month's duration, and occasionally had shortness of breath after exertion. Arrhythmia was diagnosed by the local community hospital. She was referred to our hospital for a further comprehensive examination. Her blood pressure was 119/70 mmHg. Her body temperature was 36.9°C and respiratory rate was 19 times per minute. The oxygen saturation was 98%, auscultation showed no murmur and her heart rate was about 120 bpm with regular rhythm. The electrocardiogram (ECG) showed paroxysmal SVT  can be used to visualize the anatomy of the CS and measure its blood flow, the quality of MRI may be poor due to the patient's rapid heart rate. Therefore, the preferred method of examination for this patient is echocardiography. Physicians need to be aware that CS is not included in conventional echocardiographic views, and there is the possibility of CS anatomical variation which can easily lead to missing the diagnosis. Therefore, for suspected patients, careful observation of CS is required.CECT can show the blood supply characteristics of the tumor. Bai et alUntil now, chemotherapy, radiotherapy, and surgery are the main treatments for PCL, and systemic chemotherapy is still the most fundamental therapy for the disease. In our report, the resection of CS tumor relieved patient's arrhythmia and the systemic chemotherapy prevented disease from recurring in the short follow-up time. However, the prognosis of PCL is very poor with a 10% survival rate in 9 to 12 months without any treatment, and earlier diagnosis may improve prognosis.4PCL lacks the specific clinical symptom, and various arrhythmias may be an early presentation. Surgical removal of tumor is usually indicated to avoid catastrophic complications. The utilization of echocardiography as a diagnostic modality can help in establishing the diagnosis and guide the surgical resection.The authors are grateful to all physicians contributing data to this study.Conceptualization: Xiangyu Chen.Data curation: Xiangyu Chen, Zhelan Zheng.Software: Zhelan Zheng.Writing – original draft: Ling Wang, Xiangyu Chen.Writing – review & editing: Xiangyu Chen, Lixia Cai.


Objective: This report describes a clinically rare case of congenital ectropion involving both upper lids in a one-day-old Indian newborn. We emphasize the importance of non-invasive conservative management with 5% hypertonic saline. Method: Observational case reportResult: A term newborn presented to us on day 1 with bilateral upper lid ectropion or ‘double congenital ectropion’ noted since birth following an uneventful vaginal delivery. Examination revealed severe chemosis and prolapse of upper palpebral conjunctiva bilaterally. The repeated attempts to manually revert the eyelids in position failed. Otherwise, the eyes were normal. We started to treat the baby with topical hypertonic saline (5% sodium chloride), topical antibiotic, and topical lubricant frequently. Eye pads soaked in 5% hypertonic saline were also used. Following five days of treatment, the chemosis and ectropion resolved completely without recurrence.Conclusion: We advocate non-invasive conservative management with 5% hypertonic saline soaked pads over the eyes along with topical antibiotic and lubricants. It should be the first line of treatment in all cases of congenital ectropion, before jumping to any aggressive invasive treatment like tarsorrhaphy, skin grafting etc., or unnecessary referral. Congenital eyelid ectropion is a rare condition which usually presents at birth, but late-onset cases are also noted. It was first reported in 1896 by Adams, who used the term ‘double congenital ectropion’ , 3], es, es3], eth day of treatment, the eyelids were in a normal position and reverted again if the baby cried. Following five days of treatment, the eyelids and conjunctiva were almost normal with spontaneous eye-opening. There was no recurrence thereafter even if the baby cried. The baby was discharged on the 7th day and was normal at subsequent follow-ups.Our case was a one-day-old term male neonate born to a 29-year-old Indian female. There was no significant antenatal history. The baby was born of an uneventful normal vaginal delivery and weighed 2.5 kg. He had eversion of both upper lids with chemosis, which progressively increased with crying Figure 1 . Upper pCongenital ectropion is an important entity not only because of its rarity, but also due to the panic it creates for parents and even health professionals if encountered for the first time. It usually presents at birth and is bilateral, though late-onset and unilateral cases are also reported. The exact incidence is unknown. Few sporadic cases have been reported after its first description by Adams , 4]. As. As4]. AThe exact cause of ‘double congenital ectropion’ is unknown. A case report by Young (1954) described pathophysiology to be intrinsic to some eye disease which leads to inflammatory swelling and orbicularis spasm . AccordiTreatment is largely conservative, which if started promptly gives complete recovery from this condition. Topical antibiotic eye drops and frequent lubrication are required. However, we would like to emphasize the use of hypertonic saline (sodium hydrochloride 5%) eye drops and patching the eyes with gauzes soaked in the hypertonic saline solution.The principle behind it appears to be ‘osmosis’, i.e. the hypertonic saline pulls out the fluid from the oedematous conjunctiva and thus drastically reduces chemosis. Based on the same principle, hypertonic sodium chloride ointment is used to reduce corneal oedema. We recommend the use of hyperosmolar saline patches as the first line of treatment since it is very effective and safe. The major side effects are unknown apart from mild allergic reactions, burning sensation, or irritation. In babies, an inappropriate increase in congestion of the eye should be taken cautiously and treated with antibiotics; if non-responsive, withholding hypertonic saline may be considered. Usually with this conservative approach, eyelids return to a normal position in 1–2 weeks without any after effects. Surgical intervention is needed in rare instances like non-response to conservative management, delay in conservative treatment leading to irreversible changes in the eyelids, Down’s syndrome, ichthyosis, etc. Parents need to be counseled and reassured regarding the benign nature of this condition and its complete recovery on topical medications as they can have anxiety, depression, and embarrassment. The non-surgical approach gives good anatomical and cosmetic outcomes without any sequelae if started appropriately and promptly.‘Double congenital ectropion’ is a rare condition having an alarming presentation but a benign course and excellent cosmetic outcomes after timely conservative treatment. We believe the use of 5% hypertonic saline-soaked eye pads over the eyes to be the most effective remedy. Primary health care providers – be it obstetrician, pediatrician, neonatologist or general ophthalmologist – should be aware of this condition to avoid any delay in initiating treatment and prevent any unnecessary referrals.Informed consent has been obtained from the patient’s parents for the publication of this case report.The authors declare that they have no competing interests.


Gastrointestinal stromal tumors that present outside the gastrointestinal tract are known for extra-gastrointestinal stromal tumors (EGISTs) and they share the same morphological and immunohistochemical characteristics with gastrointestinal stromal tumors. Here we report a rare case of diffuse primary EGIST arising at peritoneum.A 57-year-old male presented to the hospital with abdominal pain and right lower abdominal tenderness.The core needle puncture biopsy showed epithelial-like cells and the nuclei were ovoid and focally elongated. Immunohistochemical examination was consistent with a primary EGIST of the peritoneum.The patient was treated with Imatinib mesylate.Five months later, there is no complication resulting from treatment. The follow-up abdominal contrast-enhanced CT showed the lesion was significantly decreased in size, and was evaluated as partial response. The patient continued the treatment with Imatinib as prescribed by the oncologist.EGISTs are rare and should be considered in the differential diagnosis of the peritoneal tumors and immunohistochemistry helps to confirm the diagnosis. Further study with longer follow-up is desired to characterize these uncommon tumors. So, they are believed to represent either GISTs that were separated from the gastrointestinal tract wall,Of all the EGISTs, diffuse primary EGISTs in the peritoneum are rarely reported in past literatures. Arabi et al22.1A 57-year-old male presented with vague paroxysmal lower abdominal pain under no obvious predisposing causes. The pain lasted for a week and there was no symptom like nausea, vomiting, fever or ague. No history of bowel habit changes, fevers, or weight loss were found. The patient denied any abdominal trauma or past surgeries and his family history was unremarkable. A physical examination revealed right lower abdominal tenderness and suspicious rebound pain.9/L), monocyte percentage (12.4%), carbohydrate antigen 125 (181.3 U/ml) and C reactive protein (17.5 mg/L).Blood tests revealed increased hemoglobin (129 g/L), platelet count (312 × 102.2Contrast-enhanced computed tomography (CE-CT) was performed for initial examination. CT showed a large soft tissue mass with wide involvement of greater omentum, mesentery, peritoneum of abdomen and pelvis, but spared gastrointestinal tracts and appendix. The component was heterogeneous with high density area and peripheral contrast enhancement. Part of the lesion had nodular appearance and presented fusion of plaques. The lesion had significant enhancement after intra venous contrast administration and dilated feeding arteries could be seen near the lesion Fig. . The CT Further contrast-enhanced magnetic resonance imaging (CE-MRI) was arranged for the patients. The lesion presented isointense on T1-WI and hyperintense on T2-WI. Restricted diffusion could be seen on diffusion weighted images. A thread like hypointense capsule could be identified around the lesion on T1-WI and T2-WI . Vascular space involvement could also be seen in the lesion. Immunohistochemically, the tumor cells were positive for cluster of differentiation (CD) 117, syn, DOG-1, Vimentin, Ki-67 (≥10%) and negative for CD34, S100. The mutation detection showed mutually exclusive gain-of-function KIT platelet-derived growth factor receptor-alpha (PDGFRA) mutations. The described microscopic features, correlated with the macroscopic description, size and relative position to the digestive tract corresponds to a GIST with malignant behavior , developed outside the digestive tract, without contact to the digestive lumen. Due to the diffusive nature of current case, resection of the tumor could not be carried out and Imatinib mesylate therapy was conducted afterwards.The patient was treated with Imatinib (400 mg/qd), and experienced the mild incidence of diarrhea without other adverse effects. Five months later, the follow-up abdominal contrast-enhanced CT showed the lesion was significantly decreased in size, and was evaluated as partial response Fig. . To achi2.4Written informed consent was obtained from the patient and approval of this case study was obtained from the Institutional Review Board of the second affiliated hospital of Zhejiang university school of medicine.3α-SMA and negative for desmin and S100 protein.[EGISTs are a group of rare tumors with similar histology and immunohistochemical features to GISTs, occurring outside the gastrointestinal tract, majority of them in the omentum and mesentery or in the retroperitoneum.Although the contribution of imaging techniques (CT scan and MRI) is undeniable, preoperative diagnosis of EGISTs is difficult because the exact primary site of the process is not always easily recognized.Considering the clinical administration, surgical removal remains the standard treatment for nonmetastatic EGISTs.4EGIST is a rare tumor, and diffuse primary EGISTs of peritoneum is even more rare. When radiologists discover diffuse peritoneal lesion with focal fusion necrosis, EGIST should be considered as one of the primary differential diagnosis. Complete surgical resection is the only effective modality despite significant advances in new chemotherapeutic drugs. However, when the lesion is too diffusive to remove, targeting drugs like Imatinib might be an option. Accumulating data and extended future studies are necessary to better define EGISTs, their pathogenies, behavior and treatment.Methodology: Xiao-pei Xu.Resources: Fei Dong.Software: Fei Dong.Supervision: Jie Min.Writing – original draft: Liang-ji Lu.Writing – review & editing: Jie Min.


Duodenal obstruction is a rare event that is unlikely to be treated endoscopically. Herein, we describe the case of a 75-year-old woman who presented with vomiting and was diagnosed with food-induced duodenal obstruction. Impacted food was fragmented and removed by double-balloon enteroscopy, and the duodenal tract was reopened without any adverse events. Follow-up capsule endoscopy was performed one month after treatment to determine the obstruction etiology and it revealed a remarkably delayed passage of the capsule through the duodenum and excessive amounts of floating food residue in the third portion of the duodenum. Obstruction recurrence was not observed six months after endoscopic treatment. In conclusion, in our case, endoscopic treatment of duodenal obstruction prevented the unnecessary performance of surgery, suggesting its clinical utility for this condition. Management of an acquired duodenal obstruction is challenging because of the limited treatment options. Owing to the anatomical complexity of the area, even minimally invasive surgical procedures, such as pancreas-sparing or segmental resection of the duodenum, are associated with an 11.8%-38% rate of adverse events, including duodenal leak, fistulas and hemorrhage -6.In this report, we describe a case of food-induced mechanical obstruction in the third portion of the duodenum. Endoscopic treatment was selected instead of surgery to reopen the obstruction.A 75-year-old woman with no history of abdominal surgeries was admitted to our hospital with a chief complaint of recent-onset vomiting lasting two days. The patient had a history of type 2 diabetes mellitus and reported a history of chronic abdominal discomfort. Abdominal computed tomography (CT) revealed a mechanical obstruction with a high-density mass in the third portion of the duodenum Figure .After draining the pooled liquid in the stomach using a nasogastric tube, double-balloon enteroscopy (DBE) was used to confirm the etiology because the mass was not located within the reach of the esophagogastroduodenoscope. A food complex with a jelly-like appearance, which was not suspected to be a neoplasm or a gallstone, was impacted at the site Figure . The fooCapsule endoscopy (CE) examination was performed one month after discharge to assess the entire small bowel. No abnormalities were noted in the jejunum or ileum. A delayed passage time of 120 minutes within the duodenum accounted for 39% of the total small bowel exploration time, and food residue was observed floating in the third portion of the duodenum, suggesting that the abnormal motility of the duodenum caused food clustering, resulting in a bowel obstruction Figure . The patWhile cases of food-derived ileus have been reported, it remains a rare condition. Fourteen cases of small bowel ileus induced by rice cakes have been reported, all of which were resolved after conservative treatment . A case The etiology of the duodenal obstruction in this case is still obscure. Instead of manometry or magnetic resonance enterography, CE was used to determine the etiology in this case after confirming the absence of a small bowel stricture using CT . Not onChanges in the number of intestinal cells of Cajal and myenteric neurons due to impaired mucosal integrity and low baseline impedance in the duodenum which result in abnormal gastroduodenal motility, have been reported in patients with FD , 16. AltThis case demonstrated that endoscopic examination may aid in the treatment of ileus, with more favorable outcomes than conservative therapy. The use of DBE could help make a decision for optimal treatment in cases of ileus. Considering the anatomical complexity of the duodenum, transluminal endoscopic treatment could be a viable option to prevent the unnecessary performance of surgery. Moreover, after resolving the ileus status, CE can be clinically useful in clarifying the etiology as it provides information regarding duodenal motility as well as intra-luminal abnormality.


Angiosarcomas are soft-tissue neoplasms that originate from the vascular epithelium. The most commonly involved sites include the skin and subcutaneous tissues. In the GI tract, generally, angiosarcomas involve the spleen and liver, although locations in the small intestine and colon have been very occasionally reported. In the present study we report the unusual case of a man with duodenal epithelioid angiosarcoma, presenting with anemia and recurrent upper gastrointestinal bleeding, which was initially misdiagnosed as a Dieulafoy’s lesion. It is important to consider the diagnosis of gastrointestinal malignancy, including unusual neoplasms such as angiosarcomas, in the setting of anendoscopic appearance such as hemorrhagic nodule, purpuric mass and/or recurrent bleeding lesions that are persistent despite repeat interventions. In such cases, a biopsy should be considered to confirm the diagnosis. Angiosarcomas are extremely vascular malignancies, originating from endothelial cells that are categorized as lymphangiosarcomas and hemangiosarcomas, consistent with endothelial histology. They represent around 1-2% of all soft tissue sarcomas and 5% of all malignant skin neoplasms . GI angiHere we report the unusual case of a duodenal epithelioid angiosarcoma in a man presenting with anemia and recurrent upper GI bleeding, who was initially misdiagnosed as having a Dieulafoy’s lesion. The case is an 82-year-old male patient with a past medical history; significant past illnesses being recurrent upper GI bleeding, a stroke with residual left-sided hemiparesis, hypothyroidism, hyperlipidemia, abdominal aortic aneurysm, and hypertension presenting with melena. Over the past year, the patient had presented several times with melena, and a bleeding lesion was identified in esophagogastroduodenoscopy (EGD) in the second part of the duodenum, which was determined to be a Dieulafoy’s lesion. After the initial diagnosis of Dieulafoy’s lesion, bleeding was controlled with two clips . RecurreFurther bleeding later that week prompted embolization by interventional radiology. The patient presented this time with melena of 1-week duration. He denied any nausea, vomiting, abdominal pain, NSAID use, and significant alcohol use. He had initially presented to his primary care physician and was found to have a hemoglobin level of 6.9. He was given one unit of packed red blood cells (pRBCs) as an outpatient and was discharged home due to hemodynamic stability. Over the next two days, the patient developed worsening weakness and fatigue with continued melanic stool and presented to the emergency department with a hemoglobin level of 5.6. However, blood pressure and other vitals were stable. He received two units of pRBCs as well as IV pantoprazole in the emergency department and would receive three more throughout the admission. Endoscopy at this time revealed that the previously identified lesion had grown in size from 5 mm to 11 mm. The prior hemoclips were in place, but the lesion was still oozing. An additional clip was placed, and a biopsy was taken . On histopathology, morphologic and immunophenotypic findings strongly supported the diagnosis of epithelioid angiosarcoma . The patAngiosarcomas are aggressive mesenchymal sarcomas that originate from endothelial cells. These neoplasms, especially the cutaneous type, occur more commonly in the elderly population, in Caucasians, and are three times more likely to affect males than females. They are also known to have a propensity to metastasize and carry a poor prognosis . They reThere are four variants of skin angiosarcoma that are recognized. These include scalp and face, lymphedema associated, radiation-induced, and epithelioid angiosarcoma, with the head and neck variant being the most commonly seen. The development of these tumors has been linked to a history of radiation exposure, environmental toxins, foreign bodies, chronic lymphedema, and certain genetic factors . ChronicDue to the pervasive distribution of blood vessels and lymphatics in the body, angiosarcomas can develop in any location. Subtypes include cutaneous, visceral, and soft tissue, with cutaneous and subcutaneous tumors being the most commonly seen. Visceral angiosarcomas, as reported in this case, represent between 15% and 47% of angiosarcomas and are the most difficult to diagnose . VisceraThe GI lesions can appear in endoscopy as a centrally ulcerated nodule, hemorrhagic nodule, purpuric mass, and submucosal mass.Gastrointestinal stromal tumors (GIST), GI Kaposi’s lesions, and GI leiomyoma can be misdiagnosed as GI angiosarcoma due to a similar endoscopic appearance and clinical presentation. Endoscopic biopsies with histopathologic examination can distinguish GI angiosarcoma from other similar GI lesions . HistoloSkin angiosarcomas often present with an insidious onset of violaceous rash, or as nodules or plaques that may bleed or ulcerate in more advanced lesions . GI angiIn conclusion, as demonstrated in this case, it is important to consider the diagnosis of gastrointestinal malignancy, including unusual neoplasms such as angiosarcomas, in the setting of an endoscopic appearance such as hemorrhagic nodules, purpuric mass and/or recurrent bleeding lesions that are persistent despite repeated interventions. In such cases, a biopsy should be considered to confirm the diagnosis.The authors declare that they have no conflict of interest.


Leiomyoma is the most common benign oesophageal tumour. Half of all leiomyoma patients have oesophagus-associated complaints, such as dysphagia and epigastric pain, and the other 50% are asymptomatic with a diagnosis made on incidental discovery. Endoscopic ultrasonography is essential for an accurate preoperative workup and can enable guided-tissue acquisition for immunohistochemistry in certain cases. Smaller tumours are amenable to traditional and novel endoscopic removal in specialized centres, but some complex cases require surgical enucleation with a minimally invasive approach.An asymptomatic 60-year-old woman was accidentally diagnosed with a bifocal oesophageal mass, which was discovered by chest computed tomography. We report a rare case of a duplicated lower-third oesophageal leiomyoma, which was completely removed via the laparoscopic transhiatal approach. The patient has recovered successfully from the surgery. She has been followed up for six months with a normal oesophagram, adequate oesophageal function and no complaints observed. Pathological examination confirmed the diagnosis of leiomyoma in both lesions.To the best of our knowledge, this is the first reported case of duplex oesophageal leiomyomas removed laparoscopically. Using the minimally invasive abdominal technique, the lower oesophagus can be mobilised to the mediastinum without pleura injury and offers a good alternative to the thoracoscopic approach in patients with possible intrathoracic difficulties. At experienced centres, laparoscopic transhiatal enucleation of lower oesophageal leiomyomas and other benign tumours with a combination of intraoperative oesophagoscopy is a safe, fast and effective operation. Benign oesophageal tumours are rare, with a prevalence of less than 0.5% , while bAn asymptomatic 60-year-old woman was incidentally diagnosed with a twin oesophageal mass, which was suspected due to mediastinal widening on a routine chest radiograph. A CT of the thorax discovered the lesions with eccentric oesophageal wall thickening in the distal third of the oesophagus, causing narrowing of the lumen Fig. . GastrosBefore the scheduled operation, the patient had a history of a soft diet and received a prophylactic dose of LMWH (low-molecular-weight heparin—LMWH) a day before surgery. Third-generation cephalosporin and metronidazol were administered one hour before incision; then laparoscopic-transhiatal surgery was performed. The procedure was done under general anaesthesia with the patient in a semilithotomy, 25° reverse Trendelenburg position. Three ports were inserted in the abdominal cavity along the left costal arch, with one port placed in the epigastrium on the right side and one port, 10–12 mm in diameter, inserted directly above the umbilicus Fig. . A nasogSurgical excision (enucleation) is recommended for symptomatic leiomyomas and those greater than 5 cm. Traditionally, tumours of the middle third of the oesophagus are approached using a right thoracotomy; tumours in the distal third of the oesophagus are resected through a left thoracotomy, with all its associated morbidity . While tTo the best of our knowledge, this is the first reported case of complete laparoscopic transhiatal enucleation of twin oesophageal leiomyomas. There has been no reported case of duplex oesophageal leiomyomas removed laparoscopically or thoracoscopically to date. If located in the mediastinum, videothoracoscopic enucleation is considered the gold standard approach in patients with small-to-medium oesophageal leiomyomas . We haveIntroduced by pioneers from the Far East, rapid adoption of advanced endoscopic techniques has been observed in minimally invasive treatment for oesophageal subepithelial tumours. Using the principles of POEM , the EFTR method has spread increasingly worldwide. During EFTR, operators cut the mucosa at the level of the tumour; submucosal dissection is then performed to free the tumour. Muscle fibre dissection is carried out along the capsule to remove the lesion; then the defect is closed. There is another form of endoscopic resection called STER, which was first described by the POEM innovators from China and Japan , 24. STERobotic surgery could be another highly sophisticated treatment option in some referral centres. The procedure is safe, provides comparable outcomes to thoracoscopy and offers better ergonomics to well-trained surgeons, but only in very few, highly selected patients , 31. CerRecently, Asti et al. reported excellent perioperative and long-term results with a minimally invasive approach  in 35 patients with leiomyomas near the GEJ. They performed an additional semifundoplication, eliminating dependence on the PPI (proton pump inhibitor—PPI) in the nonendoscopic group; after a median of 49 months, no recurrences and good swallowing function were documented . Obuchi We also prefer the laparoscopic transhiatal approach for benign lower-third lesions, and its feasibility was demonstrated by our previous study with the transhiatal management of epiphrenic oesophageal diverticula . In expe


Angiomyolipoma (AML) is a common, chronic disease usually localized in kidney and liver organs; but occurring frequently outside the kidney or liver.We describe an unusual case of a 62-year-old female patient with AML in the middle ear. She presented with left earache, tinnitus and hearing loss. Preoperative computed tomography and magnetic resonance imaging seemed to reveal a middle ear cholesteatoma. The patient underwent surgical resection of this lesion, and the AML was finally confirmed by histopathological examination. The patient was discharged on the 8th postoperative day and did not seek further treatment.Extraperitoneal AML is rare and accurately identified by histopathology. The recommended management is surgery for AML in the middle ear. Angiomyolipoma (AML) is the most common benign tumour of the kidney and is related to the tuberous sclerosis complex, which is composed of vascular endothelial cells, smooth muscle cells and fat cells –3. PatieA 62-year-old female patient was admitted to the hospital in July 2019 because of left earache, tinnitus, hearing loss for 30 years and worsening earache for more than 1 month. She had not received any treatment before. The patient's personal history, past history and family history were unremarkable.Renal ultrasound, lung computed tomography (CT) and skin examination were performed in this patient to preclude the potential AML of other parts of the body outside the middle ear. On physical examination, the bilateral auricles showed no deformity or traction pain, and a large amount of purulent secretions were found in the left external auditory canal. Cerumen debris was found in the right external auditory canal, and no obvious tenderness was found in the bilateral mastoid process area. The rinne test results were as follows: left (+), right (−). The weber test result was right-sided (+). Temporal bone CT showed right otitis media mastoiditis, partial defect of the left temporal bone, no normal structure in the middle ear cavity and soft tissue thickening in the left external auditory canal Fig. . To deteThe operation was performed under general anaesthesia on the 7th day after admission. After general anaesthesia took effect, the patient was placed in a horizontal position, his head turned to the right, and the affected ear was upward. Ten millilitres of normal saline, 8 drops of pararenin and 10 ml ropivacaine were used for periauricular anaesthesia and haemostasis. The tympanic sinus was opened from the ethmoidal region, and the lateral wall of the superior tympanic cavity and the residual posterior wall of the auditory canal were removed. The facial nerve crest was ground down. The superior tympanic chamber, posterior tympanic chamber and mastoid were fused into a large cavity full of granulation. The granulation was carefully peeled off, and the facial nerve was explored. The bone canal was intact, the semicircular canal bone was not damaged, and most of the malleus and incus were rotten and lost. The residual ossicles were removed, and no residual granulation was found in the tympanum. The operation cavity was washed with normal saline, and the flap of the external auditory canal was cut and smoothed back. The operation cavity was filled with a gelatine sponge, and the incision was sutured. The granulation was sent to pathology. Intraoperatively, we observed that the mastoid bone cortex was on the fistula hole, and the majority of the bone in the posterior wall of the external auditory canal was destroyed. Moreover, the upper as well as posterior tympanum and the mastoid process were fused into one large cavity with amber, soft, and easy-to-bleed organization; most of the malleus and incus parts were rotten; the stapes could not be seen; and the facial nerve bone canal and the semicircular canal bone were intact Fig. a, b. PosAML is the most common benign tumour of the kidney, most commonly found in middle-aged women, and is composed of perivascular epithelioid cells also known as perivascular epithelioid cell tumours (PEComas) , 7. ThisThe clinical manifestations of AML lack specificity. There are no typical symptoms when the tumour is small. Patients may suddenly present local pain when the tumour bleeds internally. Haemorrhagic shock may occur when massive bleeding of the tumour occurs. In addition, purulent secretions found during physical examination may be caused by the destruction of surrounding tissues and infection by the tumour. Renal AML can be diagnosed by ultrasound, CT and MRI. However, AML in other locations is not common, and the nidus may be small, so the tumour cannot be clearly confirmed by CT and MRI. Further biopsy is needed for AML identification.The patient in this case was further aggravated by coinfection with AML. She recovered very well after operation combined with anti-infection treatment.Although this patient was coinfected and completely deaf in the left ear, there was no obvious destruction of the surrounding tissues by the tumour according to radiologic features in this case. However, if the tumour grows further, upward invasion may damage the tympanic canopy and skull base, leading to epidural abscess, purulent meningitis, and brain abscess; downward disruption may damage the jugular bulb, causing massive bleeding; disruption of the sigmoid sinus may result in thrombophlebitis or bleeding in the sigmoid sinus; anterior inferior disruption may damage the carotid artery tube, causing massive bleeding; posterior subperiosteal abscess and bezold abscess of the neck may be caused by bone defects and destruction of the mastoid tip or medial wall of the mastoid tip; and facial paralysis may be caused by disruption of the facial nerve bone canal and inflammation in the facial nerve.The radiologic examination of this patient resulted in a misdiagnosis of AML as cholesteatoma. Preoperative identification remained difficult, but surgery is able to lead a satisfactory result in our case.