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We present our single-centre experience of two cases of lipochoristoma, a rare lesion causing unilateral hearing loss. Differential diagnoses include the more common vestibular schwannoma, haemangioma and meningioma. Diagnosis was confirmed with fat suppression sequences on MRI. Management of both patients was with serial imaging, under the guidance of neuro-otology multidisciplinary team, due to the slow and typically non-aggressive progression of this lesion., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Colonic lipomas (CLs) are benign tumors of the adipose tissue of the gastrointestinal tract that are often asymptomatic. A search of medical literature in English using PubMed and Google Scholar was conducted for articles related to CL. Occasionally, patients present with intestinal bleeding or obstructive symptoms. Although intussusception is commonly observed in children, it is rare in adults. Moreover, CL as the most common entity, is very rare, with an incidence rate of 0.035% to 4.4%. Although fatty composition can assist in diagnosis through computed tomography and magnetic resonance imaging, the latter cannot exclude local infiltration. CLs are distributed evenly between both sexes and can be located anywhere in the gastrointestinal tract; however, they are more frequently located in the colon, particularly in the right colon and cecum (39.6%), followed by the transverse colon (25%), descending colon (20.8%), and the sigmoid colon (14.6%). Symptoms included abdominal pain (79.2%), alterations in bowel habits (45.8%), rectal bleeding (22.9%), colocolic intussusception (50%), weight loss (6.2%), vomiting (14.6%), and nausea (12.5%). Surgical and endoscopic techniques are widely used to manage CLs. The challenge for physicians is differentiating this lesion from malignant colonic lesions, at the outset. The risk of misdiagnosis is possible, and the recommendation in cases of doubt is still segmental surgical resection, as it ensures correct collection of lymph nodes for appropriate staging of presumed colonic carcinoma., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Terminal myelocystocele (TMC) is a rare form of spinal dysraphism which arises due to aberration in the secondary neurulation process involving the caudal cell mass. Terminal myelocystocele has been defined by Pang et al. based on essential and non-essential features. One of the non-essential features includes non dysraphic lipomas which do not tether to the neural placode. We are presenting two cases which meets all the essential criteria outlined by Pang et al. for TMC but also show the presence of a lipomatous component tethering to the neural placode, similar to a dysraphic lipoma. Through this article, we want to showcase a subset which represents "true" terminal lipomyelocystocele (TLMC), bridging the spectrum of spinal dysraphism between TMC and lipomyelomeningocele (LMM)., Competing Interests: Declarations. Competing interests: The authors declare no competing interests. Conflict of interest: None, (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Intussusception is rare in adults, making it a challenging and often delayed diagnosis. Patients may re-present with non-specific and subacute abdominal symptoms. Abdominal CT is the most sensitive imaging modality and is often required to make the diagnosis of intussusception. In contrast to the paediatric population, intussusception in adults is most often associated with a pathological lesion acting as a lead point. In adults, the lead point is often secondary to malignancy, though benign lesions can also be implicated. As such, surgical management with selective resection is the mainstay of treatment in adults. We describe a rare case of ileocaecal intussusception in an adult secondary to a large ileocaecal lipoma, successfully treated with laparotomy and resection in a remote Western Australian hospital. The increasing availability of CT imaging and skilled general surgeons in rural and remote Australia allows for the diagnosis and surgical management of such cases without inter-hospital transfer., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Suprascapular fossa lipoma extending to the suprascapular notch causing traction injury to the suprascapular nerve is a rare presentation. We report a 47-year-old male with progressive weakness of the right shoulder joint of 8 months duration, with a palpable mass over the spine of the scapula was noticed 2 months earlier and developed a sudden drop in arm following a moderate strain. A magnetic resonance imaging (MRI) scan revealed a rotator cuff tear involving the supraspinatus and infraspinatus muscles with a tumor like lesion in the suprascapular fossa, displacing the suprascapular muscle mass and extending into the suprascapular notch. Electromyography and nerve conduction velocity studies revealed suprascapular neuropathy. After histopathologic confirmation, an arthroscopic excision of the mass with decompression of the suprascapular notch was performed along with repair of the rotator cuff. Six months after the procedure, the patient had improved considerably in terms of function and postoperative MRI revealed a complete excision of the mass, and further follow-up of 2 years showed no recurrence. Suprascapular nerve entrapment can be caused by a lipoma in the shoulder, leading to weakness, atrophy, and consequent tear of the rotator cuff tendons. Arthroscopic management, after histopathological confirmation, gives good results in this situation. LEVEL OF EVIDENCE: Level IV., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Competing Interests: Disclosure All authors disclosed no financial relationships. Commentary Although subepithelial lipomatous lesions of the GI tract remain largely asymptomatic and are incidentally found during endoscopy, these lesions may lead to bowel obstruction and GI bleeding if larger than 2 centimeters. Historically, a surgical approach has been the first treatment modality because of high concern for bleeding and perforation associated with endoscopic resection. Advances in endoscopic resection techniques such as EMR and endoscopic submucosal dissection (ESD) have enabled safe resection of these lesions. The wide-base stalks of pedunculated lipomatous lesions contain the major feeding vessel; thus, preventative coagulation and tamponade of the vessel would facilitate endoscopic resection and minimize the risk of postpolypectomy bleeding. This can be achieved by placement of either a detachable snare or a hemostatic clip to “strangulate” the stalk. Blanching of the polyp is a clue for optimal placement of the detachable snare or clips at the base of the stalk. Bleeding prevention can be reinforced with epinephrine injection at the base of the lipoma. The only caveat with removing such large lesions by EMR is uncertainty with regard to en bloc resection arising from an often challenging position and limited visualization. By contrast, ESD ensures dissection of the lesion from the stalk within the submucosa, the surface capsule being kept intact. It is worth noting that the majority of lipomatous lesions are benign lipomas, but rarely liposarcoma or pleomorphic lipoma/liposarcoma can be seen. It has been hypothesized that a larger size of the subepithelial fat could lead to superficial pressure necrosis of the mucosal surface and result in ulceration and bleeding. As demonstrated in these cases, the superficial mucosa is grossly intact except areas with small ulcerations and erosions, usually towards the tip of the lesion or at the point affected the most by the bowel peristalsis. Tara Keihanian, MD, MPH, Assistant Professor, Division of Gastroenterology and Hepatology, Baylor College of Medicine, Houston, Texas, USA Amy Tyberg, MD, FASGE, FACG, Associate Editor for Focal Points

Lipomas are benign soft tissue neoplasms, involving the head and neck in roughly 15% of cases. We review a patient who presented with a palpable, anterior neck mass and progressive dysphagia, throat clearing, and dysphonia. He was found to have two simultaneous rare sites for head and neck lipomas: vallecular and intermuscular, anterior neck. He elected for surgical resection of both masses resulting in significant symptom improvement. Though originally referred for evaluation of an anterior neck mass, we underscore the importance of prompt investigation with reported laryngeal symptoms. We provide considerations for preoperative evaluation and surgical approaches. Laryngoscope, 134:3177-3180, 2024., (© 2024 The American Laryngological, Rhinological and Otological Society, Inc.)

A 54-year-old man was hospitalized with intermittent periumbilical pain for 1-month duration. Abdominal contrast-enhanced computed tomography (CT) revealed target-sign and a fat density mass measuring 2.0 × 2.5-cm in the distal ileum. Part mesenteric tissues and blood vessels were embedded and the wall of the affected intestinal tube was thickened and edematous. His symptom was alleviated after conservative treatment and he refused further management. The patient was hospitalized again with the same symptoms and abdominal CT findings 4 years later. Exploratory laparotomy was performed. A palpable mass in the ileum was found measuring 3.0 × 3.0-cm and partial enterectomy was performed. Postoperative histopathology revealed the resected mass was composed of proliferating mature adipocytes surrounded by few fibrous connective tissue. Hyperplastic fibroblast and inflammatory exudative necrotic tissue were found on the surface of the mass. The patient was diagnosed as ileum fibrolipoma with intussusception. He was discharged home uneventfully and no symptoms was observed in 12 months follow-up.

Abstract: Intraosseous lipoma of calcaneum is a rare cause of heel pain. Calcaneum is a typical site of involvement of IOL. There are only a few published articles regarding calcaneal intraosseous lipoma and one has been reported from Nepal. We report a case of 35 years female who presented with left heel pain for 1 year. The pain was gradually increasing in intensity and was severe enough to refrain her from activities of daily living. She was surgically operated with curettage and filling the defect with bone cement. There is no residual pain at 2.5 years follow up. We briefly review the postulated pathogenesis, clinical manifestations, diagnosis and various modalities of treatment of intraosseous lipoma. An orthopedic surgeon should have high degree of suspicion regarding the uncommon cause of heel pain and its possible management. When conservative methods do not relieve symptoms, surgical excision and filling the defect with bone cement provides long term relief.

Introduction: Lipomas arising in the parietal peritoneum are rare, and some of them cause abdominal pain due to torsion of the pedunculated peritoneum. We encountered a case of parietal peritoneal lipoma arising upper peritoneum. In this report, we describe the detail of clinical presentation and discuss its potential pathogenesis and treatment strategy., Case Presentation: 45 year-old Japanese female patient presented with long-lasting intermittent pain in the left upper abdominal region. Abdominal imaging showed a well-defined fatty mass measuring 40 mm in size, suggesting a parietal peritoneal lipoma. Laparoscopy revealed a tumor with a twisted peduncle; however, no adhesion of the surrounding tissues and ischemic changes were visible. The tumor was easily removed by dissection of the tumor pedicle., Conclusion: Parietal peritoneal lipoma often shows pedunculated form and it causes abdominal pain by the torsion of tumor pedicle. Therefore, this type of lipoma should be considered a more aggressive surgery., (© 2024. The Author(s).)

Introduction: Encephalocraniocutaneous lipomatosis (ECCL) is a rare congenital syndrome with complex skin, eye, and central nervous system (CNS) symptoms. Diagnosis and treatment are challenging due to its rarity and diverse manifestations. It often involves issues like porencephalic cysts, cortical atrophy, and low-grade gliomas in the CNS, resulting in developmental delays. The spinal cord is frequently affected, leading to problems like medullary compression and radiculopathy, causing back pain and sensory/motor deficits. Surgical interventions are reserved for symptomatic cases to address hydrocephalus or alleviate spinal lipomas. This article reviews a case series to assess surgical risks and neurological outcomes., Case Series: We present a case series ECCL, focusing on the diffuse lipomatosis of the spinal cord and the intricate surgical procedures involved. A multi-stage surgical approach was adopted, with continuous neuromonitoring employed to safeguard motor pathways. We discuss clinical characteristics, imaging studies, and indications for neurosurgical interventions., Discussion: ECCL is a complex syndrome. Diagnosis is challenging and includes clinical evaluation, neuroimaging, and genetic testing. Treatment targets specific symptoms, often requiring surgery for issues like lipomas or cerebral cysts. Surgery involves laminectomies, spinal fusion, and motor pathway monitoring. Thorough follow-up is crucial due to potential CNS complications like low-grade gliomas. Hydrocephalus occurs in some cases, with endoscopic third ventriculostomy (ETV) preferred over ventriculoperitoneal shunt placement., Conclusion: Neurosurgery for ECCL is for symptomatic cases. ETV is preferred for hydrocephalus, while the treatment for lipoma is based on the presence of symptoms; the follow-up should assess growth and prevent deformities., (© 2024. The Author(s).)

Intramedullary spinal capillary hemangioma is a rare occurrence in pediatric patients, and only limited cases have been reported. This study presents the first two cases of spinal capillary hemangioma co-present with retained medullary cord and one case of spinal capillary hemangioma with lumbosacral lipomatous malformation. Previous literature on ten patients with this pathology was reviewed. We speculated pathogenesis, imaging features, and histopathologic findings of the disease., (© 2024. The Author(s).)

We report the case of a 34-year-old female who was evaluated for a right lower extremity soft-tissue mass, found to be a large cystic lesion bound by fibrous tissue containing innumerable, freely mobile nodules of fat. Her presentation suggested the diagnosis of nodular cystic fat necrosis (NCFN), a rare entity that likely represents a morphological subset of fat necrosis potentially caused by vascular insufficiency secondary to local trauma. Her lesion was best visualized using MRI, which revealed characteristic imaging features of NCFN including nodular lipid-signal foci that suppress on fat-saturated sequences, intralesional fluid with high signal intensity on T2-weighted imaging, and a contrast-enhancing outer capsule with low signal intensity on T1-weighted imaging. Ultrasound imaging offered the advantage of showing mobile hyperechogenic foci within the anechoic cystic structure, and the lesion was otherwise visualized on radiography as a nonspecific soft-tissue radiopacity. She was managed with complete surgical excision with pathologic evaluation demonstrating, similar to the radiologic features, innumerable free-floating, 1-5 mm, smooth, nearly uniform spherical nodules of mature fat with widespread necrosis contained within a thick fibrous pseudocapsule. Follow-up imaging revealed no evidence of remaining or recurrent disease on postoperative follow-up MRI. The differential diagnosis includes lipoma with fat necrosis, lipoma variant, atypical lipomatous tumor, and a Morel-Lavallée lesion. There is overlap in the imaging features between fat necrosis and both benign and malignant adipocytic tumors, occasionally making this distinction based solely on imaging findings challenging. To our knowledge, this is the largest example of NCFN ever reported., (© 2023. The Author(s), under exclusive licence to International Skeletal Society (ISS).)

Competing Interests: Competing interests: None declared.

In up to 2% of the population, benign tumours called lipomas can develop. When they are more than 5 cm, they are considered giant lipomas. Giant lipomas of the distal forearm and hand may cause compression to the underlying tissues, nerves and muscles, even though they are typically asymptomatic. An older woman with soft tissue swelling in her right wrist and forearm, and numbness and pain in her right hand presented to the general surgery outpatient clinic. Her numb fingers indicated that the median nerve was compressed, and an MRI scan of her wrist and forearm revealed median nerve compression due to a giant lipoma with a dimension of about 9.2×3.4×4 cm. A surgical excision was done with an intraoperative nerve stimulator, and the specimen sent for histopathology confirmed the diagnosis of lipoma. Pain, numbness and motor power improved within 1 week postoperatively, and the patient was discharged., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Wilson's disease (WD) is an inherited disorder of copper metabolism. Agenesis of the corpus callosum is the complete or partial absence of the major united fiber bundles connecting the cerebral hemispheres. Intracranial lipoma is an adipose tissue tumor resulting from an abnormal embryonic development of the central nervous system. The simultaneous occurrence of these three disorders is rare and has not been reported. This report focuses on the pathogenesis and association between the three disorders and highlights the importance of recognizing and effectively managing their coexistence., Case Presentation: The purpose of this study was to present a patient with coexisting WD, intracranial lipoma, and corpus callosum dysplasia. We reviewed a female patient hospitalized in 2023 with clinical manifestations of elevated aminotransferases and decreased ceruloplasmin, as well as genetic testing for an initial diagnosis of Wilson's disease. Subsequently, a cranial MRI showed corpus callosum dysplasia with short T1 signal changes in the cerebral falx, leading to a final diagnosis of Wilson's disease combined with intracranial lipoma and corpus callosum dysplasia. The patient's WD is currently stable after treatment with sodium dimercaptosulfonamide (DMPS) and penicillamine, and the patient's abnormal copper metabolism may promote the growth of intracranial lipoma., Conclusion: The pathogenesis of WD combined with intracranial lipoma and corpus callosum dysplasia is complex and clinically rare. The growth of intracranial lipomas may be associated with abnormal copper metabolism in WD. Abnormal copper metabolism affects lipid metabolism and triggers inflammatory responses. Therefore, early diagnosis and treatment are beneficial for improvement. Each new case of this rare co-morbidity is important as it allows for a better assessment and understanding of these cases' more characteristic clinical manifestations, which can help estimate the course of the disease and possible therapeutic options., (© 2024. The Author(s).)

Background: Cervical lipomas accompanied by neurovascular compression are extremely rare and require surgical treatment in case of appropriate symptoms. The preferable method is gross total resection, as otherwise they tend to recur. Invasive growth is not typical for lipomas. However, large tumors can involve adjacent nerves and vessels and significantly complicate resection., Material and Methods: We present a 57-year-old patient who underwent resection of giant soft tissue cervical lipoma invading neurovascular bundle and compressing the oropharynx and esophagus with dysphagia and positional asphyxia. The patient was followed-up for previous 5 years. Resection was necessary due to tumor enlargement with appropriate symptoms. Searching for literature data was performed in the Pubmed, Medline, EMBASE, Cochrane Library and eLibrary databases., Results and Discussion: Gross total resection of 7-cm tumor was accompanied by mobilization of hypoglossal and vagus nerves, common, external and internal carotid arteries and jugular vein with repositioning of the oropharynx and esophagus. There was mild Horner's syndrome in early postoperative period. The patient was discharged in 4 days after surgery with regression of complaints. We found only 5 reports describing giant cervical lipomas invading neurovascular bundle., Conclusion: Giant cervical lipomas are extremely rare, and total resection with preservation of critical structures is possible in a specialized hospital.

Background: Lipomas are a rare cause of posterior interosseous nerve (PIN) compression. A systematic review of predictors for motor recovery has not been performed. This study sought to evaluate whether patient or lipoma characteristics are associated with motor recovery and could be used to determine when immediate tendon transfers at the time of excision should be performed., Methods: Articles describing patients with forearm lipomas resulting in PIN compression with motor weakness were included. Patient age, gender, symptom duration, laterality and largest dimension of lipoma, surgical intervention, and motor recovery were identified. Article quality was assessed via the Methodological Index for Non-Randomized Studies criteria., Results: Thirty articles reporting on 34 patients were identified. Average age was 58.2 years. Average largest lipoma dimension was 5.7 cm. All patients underwent lipoma removal, and 2 had concomitant tendon transfers. In all, 73.5% of patients had complete motor recovery at an average of 9.7 months. Patient age and largest dimension of lipoma, and duration of symptoms were not significant predictors of motor recovery. Symptom duration was a significant predictor of motor recovery in binary regression, particularly if < 18 months., Conclusions: The majority of patients with PIN weakness secondary to lipoma are likely to have complete motor recovery after excision alone. Concomitant tendon transfers should be considered for patients symptomatic for greater than 18 months. Further, adequately powered, studies are required to stratify risk factors and evaluate other modalities to identify the minority of patients who would benefit from immediate tendon transfer., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: KC is a consultant for Checkpoint Surgical. Cleveland, OH. CC, AP, SG declare that they have no conflict of interest.

Endobronchial lipoma (EL) is a rare benign tumor characterized by tracheobronchial smooth-surfaced mass, often resulting in bronchial obstruction without standard guidelines for management. This study seeks to clarify the clinical features and interventions of EL, aiming to improve its diagnosis and outcomes. A retrospective review was conducted on 28516 outpatients treated between January 2015 and December 2019 at the Department of Respiratory and Critical Care Medicine of the Second Affiliated Hospital of Air Force Medical University to collect patients diagnosed with EL. Their clinical, bronchoscopic, chest imaging, and histopathological features along with management were analyzed. Among the patients reviewed, nine were histopathologically diagnosed with EL, comprising seven males and two females. All EL patients exhibited noticeable symptoms, including cough (in eight patients), dyspnea (in six patients), fever (in three patients), expectoration (in two patients), chest pain (in two patients), hemoptysis (in one patient), and fatigue (in one patient). Chest CT abnormalities included endobronchial mass (in four patients), inflammatory exudation (in three patients), atelectasis (in three patients), and infiltration or consolidation (in two patients). In three patients, imaging showed fat density, directly leading to the diagnosis of EL. The EL lesions were distributed with six in the right lung and three in the left lung, all located within the first three subdivisions of the tracheobronchial tree. Treatment approaches varied, with one patient undergoing combined bronchoscopic resection and surgery. The remaining patients received bronchoscopic intervention such as electrosurgical snare resection, argon plasma coagulation (APC), cryotherapy, and holmium laser. Histopathological analysis confirmed the EL diagnosis. Finally, the mass removal restored bronchus patency. Taken together, EL symptoms lack specificity, necessitating reliance on histopathology for EL accurate diagnosis. Bronchoscopic interventions emerge as the preferred option for EL management, surpassing surgical approaches., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2023 Jian Chen et al.)

Competing Interests: The authors declare that they have no conflict of interest.

Competing Interests: The authors declare that they have no conflict of interest.

Fibrolipomatous hamartoma of the median nerve is an uncommon benign tumour of the childhood, which usually manifests in adolescents or adulthood with signs of compressive neuropathy at wrist. Symptomatic tumour is unusual in children below 5 years age and can be underdiagnosed. Magnetic resonance imaging provides pathognomonic features for the diagnosis, obviating the need for biopsy. Although standard ultrasonography is frequently the first-line imaging approach in the evaluation of soft-tissue masses, sonographic findings of this lesion are less frequently reported and have to be kept in mind by radiologist. We report the unusual case of carpal tunnel syndrome secondary to fibrolipomatous hamartoma of the median nerve in a 4-year-old child successfully treated with surgical carpal tunnel release., (© 2022. Società Italiana di Ultrasonologia in Medicina e Biologia (SIUMB).)

Competing Interests: The authors declare that they have no conflict of interest.

Colo-colonic intussusception is a rare clinical condition in adults. The predominant aetiology of intussusception in adults is a pathological lead point, with malignant lesions being the most common type. Lipomas are benign tumours of adipocytes that can sometimes be difficult to diagnose without histopathological confirmation as we highlight with this case report. We report a case of an asymptomatic female patient in her 50s who presented with an intussusception due to a giant colonic lipoma. Her CT imaging showed the possibility of a low-grade liposarcomatous component or atypical lipomatous tumour component. A laparoscopic right hemicolectomy was performed due to intussusception with the possibility of leading to colonic obstruction as well as diagnostic uncertainty of the risk of malignancy. Histopathology confirmed the diagnosis of a lipomatous lesion. In cases such as this, early surgical management is appropriate to rule out malignancy and prevent emergency presentation and surgery., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Closed spinal dysraphism (CSD) encompasses a heterogeneous group of spinal cord deformities, which can be accompanied by several types of skin stigmata. These skin stigmata may include inconspicuous features, such as sacral dimples and deformed gluteal clefts, but the association between such mild skin stigmata and CSD is uncertain. This study aimed to reevaluate the indication for magnetic resonance imaging (MRI) in patients with skin stigmata while considering the indication for surgery. A retrospective analysis was conducted on magnetic resonance images of 1255 asymptomatic children with skin stigmata between 2003 and 2015. Skin stigmata classification was based on medical chart data. All subtypes of CSDs except for filum terminale lipomas (FTL), FTL thicker than 2 mm or with low conus medullaris, were considered to meet the surgical indication. CSD prevalence was estimated while considering the surgical indications and assessed after excluding all FTL cases. Skin stigmata were classified into seven types, dimple, deformed gluteal cleft, hair, subcutaneous mass, appendage, discoloration, and protruding bone, and included 1056 isolated and 199 complex ones. The prevalence of CSD was 19.5%, 6.8%, and 0.5% among patients with isolated dimples (n = 881) and 13.9%, 5.8%, and 0.7% among those with isolated deformed gluteal clefts (n = 136) for all cases, surgical indications, and patients without FTL, respectively. Dimples and deformed gluteal clefts had a low prevalence of CSD requiring surgical intervention, and cases without FTL were rare. Asymptomatic patients with mild skin stigmata may not require immediate MRI.

We present a case of a man in his 80s with an incidental posterior cerebral artery aneurysm encased within a lipoma. The literature surrounding the incidence and intricate relationship of lipomas to cerebral aneurysms is reviewed. Lipomas are proposed to be derived from maldifferentiated subarachnoid space. For this reason, lipomas are often associated with vascular malformations and may develop in conjunction with vascular malformations such as cerebral aneurysms. Hypothesised theories include the impediment of smooth muscle nutrient diffusion and the secretion of factors that weaken the arterial wall thereby predisposing to aneurysm formation. When lipomas neighbour cerebral vasculature, careful evaluation of the adjacent vessels should be conducted., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

The technical evolution of the surgery for spina bifida occulta (SBO) over the course of a half-century was reviewed with special foci placed on the spinal lipoma and tethered spinal cord. Looking back through history, SBO had been included in spina bifida (SB). Since the first surgery for spinal lipoma in the mid-nineteenth century, SBO has come to be recognized as an independent pathology in the early twentieth century. A half-century ago, the only option available for SB diagnosis was the plain X-ray, and pioneers of the time persevered in the field of surgery. The classification of spinal lipoma was first described in the early 1970s, and the concept of tethered spinal cord (TSC) was proposed in 1976. Surgical management of spinal lipoma with partial resection was the most widely practiced approach and was indicated only for symptomatic patients. After understanding TSC and tethered cord syndrome (TCS), more aggressive approaches became preferred. A PubMed search suggested that there was a dramatic increase of publications on the topic beginning around 1980. There have been immense academic achievements and technical evolutions since then. From the authors' viewpoint, landmark achievements in this field are listed as follows: (1) establishment of the concept of TSC and the understanding of TCS; (2) unraveling the process of secondary and junctional neurulation; (3) introduction of modern intraoperative neurophysiological mapping and monitoring (IONM) for surgery of spinal lipomas, especially the introduction of bulbocavernosus reflex (BCR) monitoring; (4) introduction of radical resection as a surgical technique; and (5) proposal of a new classification system of spinal lipomas based on embryonic stage. Understanding the embryonic background seems critical because different embryonic stages bring different clinical features and of course different spinal lipomas. Surgical indications and selection of surgical technique should be judged based on the background embryonic stage of the spinal lipoma. As time flows forward, technology continues to advance. Further accumulation of clinical experience and research will open the new horizon in the management of spinal lipomas and other SBO in the next half-century., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Lipomas are common benign neoplastic mesenchymal tumours arising from adipose tissue anywhere in the body. Giant intra-thoracic lipoma extending across the anterior mediastinum into bilateral pleural spaces causing compression of the underlying lung resulting in respiratory embarrassment is rarely seen and reported. Giant lipomas of thorax require surgical excision as they continue to grow and cause compressive symptoms.

Background and Purpose: Non-dysraphic intramedullary spinal cord lipomas (NDSCL) represent 1% of spinal cord tumors. They are less frequent than dysraphic spinal cord lipomas and clinical presentation is unspecific. There are no guidelines on surgical management., Material and Methods: We report three observations of NDSCL in children, focusing on the clinical presentation, surgical management and postoperative outcome., Results: The patients, one female and two males, aged from 5 months to 10 years presented with neurological deterioration, pain, spinal rigidity and in two cases, a subcutaneous mass. Spinal MRI found intradural lipomas without spina bifida, located in the cervico-thoracic area in all cases. The lipoma extended to the medulla oblongata in two cases and was in the lumbar region in the third. These lipomas were massive, requiring decompression surgery. Surgery confirmed the lipoma to be subpial. We performed debulking of the lipoma without attempting total resection, and with or without dural plasty and laminoplasty, followed by minerva cast in two cases, and avoidance of standing in the youngest. Satisfactory recovery occurred in all three cases. After a follow-up between 4 months and 9 years, the outcome was favorable in all cases, and no patient presented with secondary spinal deformation or lipoma progression., Conclusion: NDSCL is a rare entity, which often manifests with progressive pain and neurological deficits. In our experience, partial resection with or without dural plasty and laminoplasty has been associated with satisfactory postoperative outcomes and no recurrence of symptoms. We should be attentive to the risk of postoperative spinal deformity in these young patients., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Objective: To characterize the clinical manifestations and genetic basis of a familial cancer syndrome in patients with lipomas and Birt-Hogg-Dubé-like clinical manifestations including fibrofolliculomas and trichodiscomas and kidney cancer., Methods: Genomic analysis of blood and renal tumor DNA was performed. Inheritance pattern, phenotypic manifestations, and clinical and surgical management were documented. Cutaneous, subcutaneous, and renal tumor pathologic features were characterized., Results: Affected individuals were found to be at risk for a highly penetrant and lethal form of bilateral, multifocal papillary renal cell carcinoma. Whole genome sequencing identified a germline pathogenic variant in PRDM10 (c.2029 T>C, p.Cys677Arg), which cosegregated with disease. PRDM10 loss of heterozygosity was identified in kidney tumors. PRDM10 was predicted to abrogate expression of FLCN, a transcriptional target of PRDM10, which was confirmed by tumor expression of GPNMB, a TFE3/TFEB target and downstream biomarker of FLCN loss. In addition, a sporadic papillary RCC from the TCGA cohort was identified with a somatic PRDM10 mutation., Conclusion: We identified a germline PRDM10 pathogenic variant in association with a highly penetrant, aggressive form of familial papillary RCC, lipomas, and fibrofolliculomas/trichodiscomas. PRDM10 loss of heterozygosity and elevated GPNMB expression in renal tumors indicate that PRDM10 alteration leads to reduced FLCN expression, driving TFE3-induced tumor formation. These findings suggest that individuals with Birt-Hogg-Dubé-like manifestations and subcutaneous lipomas, but without a germline pathogenic FLCN variant, should be screened for germline PRDM10 variants. Importantly, kidney tumors identified in patients with a pathogenic PRDM10 variant should be managed with surgical resection instead of active surveillance., Competing Interests: Declaration of Competing Interest The authors have nothing to declare., (Published by Elsevier Inc.)

Objective: Lipomas are very common tumors which usually prefer the upper limbs and, depending on their size, may cause nerve compression, or may be asymptomatic. The current cadaveric report describes a giant lipoma in the distal forearm area., Case Report: A large mass (5.1 × 3.2 × 1.6 cm) was identified on the palmar surface of the distal forearm, during dissection of a 63-year-old male cadaver. The mass caused anteromedial displacement and flattening of the median nerve (MN). Despite the lack of information about the subject's medical history, MN compression was assumed on the basis of the lipoma's size, its vicinity to neural structures, and the MN displacement and flattening., Conclusion: The enlarged distal forearm lipoma, located adjacent to the carpal tunnel, displaced and flattened the MN. The cadaveric finding described is clinically relevant for both differential diagnosis and surgical treatment of carpal tunnel syndrome., (Copyright © 2023 Natsis et al. This article is available under a Creative Commons License (Attribution 4.0 International).)

Abstract: Intracranial lipomas are extremely rare fat-containing lesions that occur due to the abnormal differentiation of embryonic meninges. Interhemispheric lipomas that are closely related to the corpus callosum are also referred to as pericallosal lipomas and are the most common subtype. Pericallosal lipomas are associated with varying degrees of dysgenesis of the corpus callosum and therefore have a broad clinical presentation. Herein, we describe a case of a 15-year-old man who presented with a history of developmental delay and psychiatric symptoms and was incidentally found to have a pericallosal lipoma on magnetic resonance imaging, prompting clinical correlation., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Variations in split cord malformation (SCM) are known. However, association of SCM type I with myelomeningocele along with same level dorsal bony spur has not been described previously. We report a 1-year old male child who presented with these findings with associated syringomyelia, lipoma and tethered cord.

Background: Lipomyelomeningoceles (LMMs) are subcutaneous lipomas with dural penetration that often present with spinal cord tethering and may lead to neurological deterioration if untreated. This report describes a rare case of an LMM associated with immature nephroblastic tissue, representing a nephrogenic rest (NR) or, less likely, an immature teratoma., Clinical Presentation: An 8-day-old infant girl presented to the clinic with a sacral dimple. Imaging demonstrated a tethered spinal cord with low-lying conus medullaris and an LMM. A firm mass was noted in the subcutaneous lipoma. Detethering surgery and removal of the lipoma and mass were performed at the age of 6 months. Pathological examination identified the mass as cartilage, fat, and immature nephroblastic tissue consistent with NR tissue or, less likely, a teratoma with renal differentiation., Conclusion: This presentation of an LMM associated with an immature teratoma or NR poses a risk of malignant transformation in patients. As a result, careful surgical dissection, resection, and close clinical follow-up are recommended for these patients., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

The reporting case is a 27 year-old woman. She was admitted with complaints of clumsy gait and dysesthesia over her upper limbs and spasticity in the legs. The investigations revealed a long segment cervical intramedullary tumor, associated with whole cord syringomyelia. The superior part of syringomyelia image intensity matched intramedullary lipoma completely. C1-C5 intramedullary tumor was resected grossly under microscopic view, and a fat-containing liquid exited the syringomyelia cavity at its superior pole. After a transient paresis just following the surgery, neurological condition was improved. The follow-up imaging showed that the fatty syringomyelia reduced in dimensions. Conclusion . According to this case and another similar case due to instability, it appears that as a rare phenomenon, syringomyelia liquid content sometimes could have fatty droplets which could mimic intramedullary lipoma with an unknown mechanism. It seems these fat droplets in syringomyelia would not change its natural history.